No

Case Report:

A 33 year-old ASA1, primigravida, presented to our delivery suite with spontaneous onset of labour at 38 weeks of gestation. Epidural analgesia was commenced to alleviate her labour pains. Subsequently, she underwent an assisted vaginal delivery of a live male baby (weighing 4660 gms) using Keiland’s outlet forceps after 90 min second stage of labour. 10 hours postpartum, she complained of dyspnoea & severe central substernal chest pain. She was noted to have an unusual swelling of face and neck with oxygen saturations of 90 % on room air. Ascultation of chest revealed normal bronchovesicular breath sounds, normal heart sounds with absence of added sounds. Arterial blood gases showed an O₂ tension of 11 kpa, CO₂ tension of 5 kpa and pH of 7.34. The diagnosis of subcutaneous emphysema, pneumomediastinum and small left apical pneumothorax (Hamman’s syndrome) was confirmed on chest X-ray (CXR 1). We ruled out differential diagnosis of pulmonary embolism, Tension pneumothorax, angina pectoris, pericarditis, dissection of aortic aneurysm, mediastinitis, cardiac tamponade, chest infection & oesophageal tear. She was managed conservatively by close monitoring for complications, administration of supplemental oxygen and use of simple analgesics. She demonstrated a complete uneventful recovery over the next 24 hours with normalising of chest signs (CXR 2).

CXR 1: shows pneumomediastinum, extensive surgical emphysema & a left apical pneumothorax.

CXR 2: shows small pneumomediastinum, the surgical emphysema & pneumothorax resolved.

Discussion:

Hamman’s syndrome is named after Louis Hamman (1847-1946), the physician who first described it in 1945. The first reference to this condition was in 1618, when Louise Bourgeois, midwife to the Queen of France, wrote, “I saw that she tried to stop crying out and I implored her not to stop for the fear that her neck might swell”³_.

Hamman’s syndrome usually occurs in the 2^ndstage of labour & is associated with prolonged and protracted labour and larger than usual babies ⁴_. However, the clinical presentation is often delayed to the postpartum phase as was clearly seen in our case. The condition seems to be provoked by any valsalva manoeuver such as vigorous coughing/vomiting/sneezing, forced physical activity & enormous efforts during spontaneous vaginal delivery. Its occurrence is usually related to the expulsive phase of labour when ‘pushing down’ actively raised the intraalveolar pressure. This may subsequently increase the intrathoracic pressure up to 50 mm of Hg or higher¹. Rupture of marginal alveoli with air entering along the perivascular sheath into the mediastinum is the most likely mechanism, in our case. It is probable that, the air tracts through the fascial planes into subcutaneous and retroperitoneal tissues. Other reported mechanisms of Hamman’s syndrome include oesophageal rupture during childbirth, or pneumomediastinum related to asthmatic bronchospasm⁵ or chest infection, or dissection of pneumoperitoneum, secondary to epidural catheter placement or caesarean section^1.

Palpable crepitus on face & neck is suggestive of subcutaneous emphysema & appearance of this emphysema in labour is the hallmark of pneumomediastinum. Other features of pneumomediastinum include substernal chest pain, dyspnoea, voice change, cough, sore throat and tachycardia¹. Hamman’s sign, a fine auscultatory crepitation synchronous with the heartbeat, heard along the left sternal border; is sometimes observed in this condition².

Chest X-ray and CT thorax are the diagnostic tests. Majority of the patients with Hamman’s syndrome have pneumomediastinum & subcutaneous emphysema without any pneumothorax and this requires supportive management with strict monitoring. Our patient demonstrated a small pneumothorax, which was managed conservatively. A surgical intervention in the form of subcutaneous air drainage may occasionally be indicated in severe cases.

Overall most cases have a benign, self-limiting course when the aggravating factors are no longer present. Published data indicates that subsequent pregnancies pose no additional risk of recurrence⁵.

Conclusion:

Since Hamman’s syndrome is a potentially dangerous complication of normal childbirth. We propose that every obstetric anaesthetist and obstetrician should be aware of this syndrome.

Introduction

Fractures in surgically fused scoliotic spines are very uncommon and only a few cases have been reported in the literature. It is not possible to predict the outcome of traumatic injuries in fused spines. There is no reported prevalence or prognostic data in the published literature and all we have are a few case reports from different parts of the world.

In this case report we describe an unusual case of a spinal fracture in a 60-year-old patient, who had surgical fusion of her scoliotic spine 50 years ago.

Case Report

A 60-year-old lady presented to A&E after a trivial fall on an icy path approximately 10 days before presentation. She had pain in her back since the fall, gradually getting worse despite escalating doses of opiate analgesics. Past medical history revealed that she had congenital lympho-haemangioma causing deformity in her back and left foot. At the age of 6 months she underwent an extensive surgical excision of the tumour along with amputation of her left foot. Subsequently she developed scoliosis at the age of 6 years, which was treated conservatively in a Milwaukee brace between the ages of 7 and 10. At the age of 10 she underwent an extensive thoraco-lumbar postero-lateral inter-transverse fusion using iliac crest bone graft without instrumentation to treat her progressive scoliotic curve. She was supported in a Milwaukee brace for further 6 months. Following this she had no problems in her back although she had a considerable residual deformity.

After this recent fall she developed pain in her right-sided thoracic hump. A full neurological examination revealed normal motor & sensory function in both lower limbs. Plain radiographs showed a thoracic scoliosis convex to the left and a broad fusion mass extending approximately from T4 to L1. There was no fracture seen. She was discharged from the A&E department with further analgesia. 3 days later she returned to A&E with increasing pain and respiratory depression due to excessive opiate usage. Investigations also revealed a very high level of serum lithium from her regular lithium medication combined with dehydration and deranged renal function. She was admitted in the high dependency unit for supportive care while the symptoms of pain and discomfort were progressively worsening. Another radiograph of her spine was again inconclusive of any bony injury. A CT scan was performed at this juncture. The CT scan (Fig. 1) showed a fracture line at the junction of T9-T10 extending through the fusion mass, with minimal displacement. She was neurologically stable on clinical examination.

The feasibility of surgical fixation of this fracture was discussed with a specialist scoliosis surgeon and a decision was made to pursue conservative treatment, considering her ongoing medical condition. Surgical fixation was deemed to be technically challenging and very risky. She was not found to be suitable for bracing either. She was advised bed rest with symptomatic management of pain, which was followed by protected and supervised mobilisation.

Further CT scans were performed after 6 weeks and after 12 weeks. These showed the fracture had remained stable but minimal signs of healing were observed with persistent gas shadows in the disc space. Throughout this she remained free of any neurological deficiency and her pain was under control. She was allowed mobilisation within the limits of comfort and under supervision. Serial CT scans were performed at the 7^th month, which showed a stable spine and some callus formation at the fracture site. The latest follow up scans performed at the 12^th month showed bony union had taken place (Fig 2 & 3). She was followed up in the outpatient clinic. She has resumed her normal activities and is now not requiring regular analgesia.

Figure 1- Coronal and Saggital CT Images of the Fracture from January 2011

Figure 2- Coronal Images compared between July 2011 and February 2012 showing healing

Figure 3- Saggital Images compared between July 2011 and February 2012 showing healing

Discussion

Fracture through a fused scoliotic spine is an uncommon entity. Healing of that fracture by conservative measure is fairly uncommon. Most authors point out that “the ankylosed spine breaks like a long bone, transversely, as a result of a bending force” (Bergmann)¹. This fracture configuration results in higher rates of non-union and delayed union. In this light we have presented here a unique case report where a fractured fusion mass has healed without surgical intervention.

There are very few reported incidences of fracture through a spinal fusion mass after scoliosis surgery in the published literature in English. Two patients reported by Moskowitz et al² had injuries as a result of traffic accidents. The exact mechanisms of the injuries were not described and their management was not discussed. One fractured through the fusion mass 20 years after surgery, the other 14 months after surgery.

King and Bradford³ described a fracture-dislocation of T11-T12 in a patient treated with Harrington rods. They decided to operate because of rod angulation and severe trunk de-compensation.

Tuffley and McPhee⁴ described a patient treated with posterior spinal fusion without instrumentation. The patient sustained a transverse fracture through the fusion mass without displacement after a fall. Posterior fixation with Harrington instrumentation was carried out.

Bagó et al⁵ described a 30 year old woman who had undergone anterior and posterior fusion with Cotrel-Dubousset instrumentation for progressive idiopathic scoliosis. Two years after surgery, she was in a car accident. A radiographic study and computer tomographic scanning depicted a fracture of T11 and bending of the rods. Observation was instituted and symptoms resolved.

Chung⁶ reported a post polio patient whose spine was fused from T7 to L4 as a teenager by spinal instrumentation, which was removed after achieving fusion. She fell down stairs fracturing the body of L2 without any neurological deficit. She was treated conservatively for 3 months after which non-union was observed. The fusion mass was fixed with an AO/ASIF broad dynamic compression plate rather than the convention spinal systems such as pedicle screws, Harrington or Luque system because of the absence of normal anatomical landmarks.

All the described case reports were from high-energy trauma unlike our case where the injury was very inconspicuous. We stress upon the fact that these injuries are very rare and can be very difficult to diagnose from plain radiographs. Our patient was fortunate not to have damaged her spinal cord, which is probably because of the low energy trauma she sustained. Our conservative management has worked well in alleviating her symptoms and achieving bony union.

Introduction

Medical Student Syndrome (MSS) is a unique type of hypochondriasis which specifically causes health anxiety related to the diseases medical students study during their medical training.¹ However, this phenomenon does not translate into an increased number of consultations differentiating it from hypochondriasis.² Nevertheless, the common denominator in both conditions is that the affected person persistently experiences the belief or fear of having severe disease, due to the misinterpretation of physical symptoms.³ The medical examination on multiple occasions does not identify medical conditions that fully account for the physical symptoms or the person’s concerns about the disease, making it a diagnosis of exclusion. Unfortunately, the fears frequently persist among medical students despite medical reassurance, affecting their concentration during their training.⁴

Earlier studies have shown a higher prevalence of MSS in various medical schools, but recent studies show a declining trend. While Howes et al⁵ demonstrated that 70% of medical students have groundless medical fears during their studies, Weck et al,⁶ on the contrary, recorded the prevalence of health anxiety only among 5-30 % of study participants. One of the reasons ascribed to this could be that earlier studies, showing a high prevalence of MSS, were uncontrolled. Also, age-matched peers were not used as controls in some studies, and no direct interviews had been conducted.^7,8 Methodological issues in previous data have led to inaccurate interpretations and over-generalization of findings. For example, the high emotional disturbance in medical students resulted from comparisons made with the general population, rather than with other students of their age. ^9-11

We were prompted to conduct this study because the magnitude of MSS is variable from region to region, and in this study we compared medical students with their peers, studying in different colleges of Taif University to avoid observational bias.

Methods

This study was carried out from September 2017 to June 2018 at the female campus of Taif University, Kingdom of Saudi Arabia (KSA) in medical (pre-clinical and clinical years) and non-medical colleges in accordance with research guidelines of the College of Medicine, Taif University, KSA.

Inclusion criteria

Age and gender-matched students were selected for inclusion in the study. These included:

1. Female medical students from the second to the sixth grades enrolled in the College of Medicine, Taif University, KSA.

2. Female non-medical students from first to fourth grades enrolled in colleges of Arts, Admin and Financial Sciences, Computer and Information Technology, Science and Islamic Law.

Exclusion criteria

Biology students were excluded due to the medical content of their courses. At the time of enrolment, permission for participant recruitment was obtained from the concerned faculty administrators.

The participants were approached in the common/study rooms or lecture halls. The students were informed of the voluntary nature of the participation and were randomly selected. They were not required to provide their names during completion of the questionnaire and were assured of confidentiality. The Hypochondria/Health Anxiety Questionnaire (HAQ), developed by the Obsessive Compulsive Centre of Los Angeles (http://ocdla.com/hypochondria-test), was used to collect the data. The questionnaire was translated into Arabic and underwent a revision in order to ensure compatibility with the original one. The questionnaire was not designed to provide a formal diagnosis but provided an indication as to whether or not the persons were exhibiting significant signs of the disease.

Results of this questionnaire were analyzed as under:

A) 1 to 3 test items checked: there is a low probability that the student has health anxiety, and it is unlikely that her concerns significantly impact his life.

B) 4 to 7 test items checked: there is a medium probability that she has health anxiety, and a moderately high amount of distress related to specific health-related thoughts. She spends more time than most people doing unnecessary behaviours related to these thoughts.

C) More than 7 test items checked: there is a high probability that she has health anxiety. She most likely has a significant amount of distress related to certain health-related obsessions, and likely spends a significant amount of time doing unnecessary compulsive and avoidant behaviours directly related to these obsessions.

Statistical methods

Data were statistically described regarding frequencies (number of cases) and valid percentages for categorical variables. The response of the two groups was analyzed by student t-test. P values less than 0.05 were considered to be statistically significant. All statistical calculations were done using computer program IBM SPSS (Statistical Package for the Social Science; IBM Corp, Armonk, NY, USA) release 21 for Microsoft Windows.

Results

400 students were included in the study. There were 200 medical students, and the other 200 students were from various non-medical colleges of Taif University (Colleges of Arts, Admin and Financial Sciences, Computer and Information Technology, Science and Islamic Law).

All participating students were females (100%), and the mean age of the medical students was 21 years (ranged from 19-22years). The mean age in the non-medical group was 20.5 years (ranged from 19-23 years).

All students in the non-medical colleges completed the HAQ while five students in the medical college (clinical years) did not complete it, so the data on 395 participants were finally analyzed.

According to the scaling criteria, this study showed that the overall prevalence of MSS among the total sample (medical and non-medical female students) was 16.2% (64 out 395 students). However, it was higher in the medical students (34 out of 195 students; 17.4%) than in the non-medical students (30 students out of 200; 15%) – see Table 1.

Table 1. The frequency of Medical Student Syndrome (MSS) among medical and non-medical students.

Figure 1. The difference of Medical Student Syndrome (MSS) between pre-clinical and clinical years (p=0.028).

Figure 2. Fears related to diseases in the study cohort.

While comparing the response of the two groups by student t-test, there was no statistically significant difference between the responses obtained from medical and non-medical colleges (p=0.31). However, from the MSS diagnosed cases in the medical college, there was a significant difference between pre-clinical and clinical years – 21.1% vs 14% (p= 0.028) – see Figure 1.

Regarding the percentage of students who visited the doctors during the last year due to fears from disease, or medical condition, it was higher in the non-medical student's group than in the medical student's group with a significant difference observed (p=0.043).

The medical conditions that caused worry among medical and non-medical students were, diabetes mellitus followed by cancers especially breast cancer. The least worried diseases were headache and heart diseases – see Figure 2.

Regarding the percentage of students who consulted more than one doctor for the same medical concern, because of doubt about the previous doctor’s diagnosis and laboratory results, it was higher in the non-medical student's group compared to the medical student's group. The difference was significant (p=0.001).

The students with MSS in the total sample (of 395 students) were categorized according to the degree of probability into low, medium and high as shown in Figure 3.

Figure 3. The probability of Medical Student Syndrome (MSS) among all groups compared to their non-medical peers.

Discussion

The unrealistic fears about illnesses recorded in this study among medical students were higher than their peers studying various non-medical courses at Taif University; however, the difference was not significant. The subgroup analysis revealed a correspondingly higher prevalence of health anxiety during pre-clinical years than clinical years as shown in Figure 1. Possibly during the pre-clinical years, students have an increased sense of body awareness and stress as demonstrated by Moss-Morris et al.⁷ The authors in the above study described this syndrome as a normal perceptual process and differentiated it from common hypochondriasis. Other researchers ^8,12 as well affirmed this. Our results are in parallel with the finding of Azuri et al¹³ who recorded that first-year students visited a general practitioner (GP) or specialist more often than in other years. The authors in the above study suggested that the pre-clinical students` visits may be due to registering with a new doctor closer to university or due to necessary health checks before the beginning of their medical school. The dream content of pre-clinical medical students frequently involved a preoccupation with a personal illness of the heart, the eyes and the bowels in the above study.

Additionally, the fear of acquiring a future disease is a core feature of health anxiety, while fear of already having a disease is considered more central to the MSS.¹⁴ There is a number of instances where this syndrome manifests among students from time to time during their training. The students are even known to change their diagnosis depending upon their clinical rotation. For example, in a psychiatry rotation the student conceptualizes having schizophrenia and later shifts his or her diagnosis to Meniere's disease during an ear, nose and throat (ENT) rotation. The symptoms are thought to occur due to intensive exposure to knowledge affecting symptom perception and interpretation.¹⁵ The fact remains that the affected student is devoid of either. At times, the simple knowledge of the location of the appendix transforms the most harmless sensations in that region into symptoms of a serious threat.¹⁶ The students who study "frightening diseases" for the first time routinely experience intense delusions of having the disease, reflecting a temporary kind of hypochondriasis.¹⁷

In a study by Waterman et al¹⁸ it was observed that 80% of medical students conceptualize diagnoses ranging from tuberculosis to cancer while studying these diseases during training. This caused emotional distress and conflict in them. It was suggested that this phenomenon was present in approximately 70-80% of students in the study mentioned above. There may be multiple reasons for precipitation of this condition among medical students. The vastness of medical studies are undebatable, and medical schools cause students to experience a large amount of psychological pressure due to work required to grasp the subject matter, the stress of examinations, and the competitive environment.¹⁹

In this study, we compared medical students with the students of the same age and gender with the same cultural background in order to avoid any bias. Our results are in parallel with a more recent study, which compared three groups, medical students, non-medical students, and their peers who were not undergoing any academic course. The authors in the study mentioned above observed no significant differences between the groups on total scores in the questionnaires. However, when considering the individual components of the questionnaires, it was found that medical students were less aware of bodily changes and sensations than the other groups; nevertheless, they did not avoid seeking medical advice for any health-related fears.²⁰

Regarding the percentage of students who visited doctors in the past 12 months due to fear of disease, it was observed in this study that the non-medical group had significantly higher visits to doctors compared to their peers studying in the medical college of the university. It is entirely possible that they had increased access to personal advice from peers, relatives, and various mentors. Of the various diseases, fear of diabetes mellitus was the highest, possibly due to a high prevalence of the disease in Saudi Arabia.²¹ Further, it is entirely possible that medical students subconsciously conceive these metabolic disorders as these are discussed in greater details during their courses.

MSS may lead to cyberchondria, a phenomenon of the public, seeking to diagnose themselves via the internet,¹¹ which in turn may lead to hypochondriasis in any given student. Thus, it becomes imperative that students suffering from this disorder must be dealt with an empathetic approach and counselled properly after ruling out an organic cause of their illness. A step to circumvent it further would be that MSS must be thoroughly discussed among medical students during their training.

Limitation of the study

The drawback of this study is that that the questionnaire was translated from English into Arabic, and although it underwent a revision, there were no other formal tests such as linguistic and cultural validation to validate the translated version. Further, we believe that our focus was only on female students, and it is well known that females have better ability to cope up with anxiety and depression compared to males^22,23 so the figures of MSS among male medical students needs to be studied as it may be different from what we reported in this female cohort.

Conclusion

In conclusion, the students who are suffering from MSS often overuse medical resources and outpatient’s services compared to others. Therefore, clinicians should be aware of these students, to avoid unnecessary procedures and treatments. However, it is vital that a proper evaluation is done before labelling a given student with MSS.

Background

Necrotising soft tissue infections (NSTI) are severe and rapidly progressive, requiring rapid recognitions and early, often surgically-based, management. Mono-microbial types of NSTI (i.e. Type 2 NSTI), which amounts for 20 to 30% of overall cases, are often linked to invasive Group A Streptococcus or Staphylococcus Aureus infections ¹. Rarely, Group B Streptoccocus (GBS), also known as Streptococcus Agalactiae, are implicated ². We report a unique case of NSTI of the lower limbs due to GBS, with acute pericardial dissemination leading to cardiac tamponade, leading to a diagnostic dilemma due to co-existing cardiogenic and septic shock.

Case Report

A 51-year old gentleman of Chinese ethnicity presented with right foot pain and swelling over 2 weeks, associated with chest pain and shortness of breath during that period. He had a 10-year history of poorly controlled diabetes mellitus with a Hba1c level of 8.8 %, hypertension and dyslipidaemia.

He was hypotensive on arrival, with a blood pressure of 91/60 mmHg and hypoxic, requiring high flow oxygen of 15L/min to maintain saturations at 100%. Otherwise other vitals were stable, pulse rate being 72 beats per minutes, respiratory rate 24 breaths per minute and a temperature of 37.4 degrees Celsius.

Clinical examination revealed a gangrenous lateral two toes extending into the lateral malleolus on the right foot, with evidence of pus discharge and associated warmth and crepitus up to hindfoot level on palpation. There was also evidence of dry gangrene in the fourth toe of the left foot, with presence of a small puncture at dorsum of foot with pus discharge. Similarly, crepitus was felt up to midfoot level on palpation of the left side. Bilateral dorsalis pedis and posterior tibialis pulses were palpable but feeble.

Table 1: Blood Investigations on Admission

Figure 1a & 1b: Radiography of the (1a) left foot and (1b) right foot respectively, demonstrating gas within soft tissue bilaterally.

Figure 2: Chest radiography demonstrating cardiomegaly and globular-shaped heart, with loss of left-sided cardiophrenic and costophrenic angles.

Figure 3: Electrocardiogram demonstrating widespread saddle-shaped ST-elevation, consistent with pericarditis.

Figure 4: Parasternal Long Axis view of bedside echocardiography showing evidence of pericardial effusion and right atrial and ventricular collapse

Figure 5: Purulent pericardial aspirate via pericardiocentesis

Initial blood investigations are highlighted in Table 1. HIV Antibody, Hepatitis B Surface Antigen and Hepatitis C Antibody serology were all negative. Lower limb radiography revealed evidence of gaseous shadows bilaterally (Figure 1). The clinical and radiological findings were consistent with necrotising soft tissue infection of bilateral feet, and the patient was advised for extensive wound debridement and possible amputation of the affected sites during an orthopaedic consult.

However, on closer review of the chest radiography, there was evidence of cardiomegaly with a globular-shaped heart (Figure 2). His electrocardiogram on arrival, revealed diffuse ST-segment elevations on majority of leads, ST-segment depression on lead aVR consistent with pericarditis (Figure 3).

A bedside echocardiogram was performed, revealing a massive pericardial effusion, measuring largest at 2 cm in depth, with evidence of both right atrial and ventricular collapse (Figure 4).

An urgent pericardiocentesis was performed, under echocardiographic guidance, which revealed turbid-looking aspirate (Figure 5). Urgent microscopic analysis revealed 45 Cells per mm³, majority of which were lymphocytes, and gram stain showed moderate amounts of pus cells with occasional gram positive cocci. Pericardial fluid was negative for acid-fast bacilli.

A repeat transthoracic echocardiogram was performed post-pericardial drain insertion, revealing minimal remnant pericardial fluid, with the pericardial drain in-situ, and no evidence of any mass or vegetation. Unfortunately, a transoesophageal echocardiography and Computed Tomography (CT) imaging of the mediastinum (to rule out mediastinitis and pneumonitis) was not performed, as management of the NSTI took precedence.

The patient was started on intravenous antibiotics, both tazobactom-pipericillin and clindamycin. There was a delay in performing limb saving wound debridement as the patient was reluctant for invasive management, but had later consented to the procedure which was performed only on day 3 of admission. Tissue cultures were taken peri-operatively. Unfortunately, the patient deteriorated post-operatively due to extensive blood loss and overwhelming septicaemia and succumbed to his illness 72 hours after. Subsequently, it was revealed that cultures from blood, pericardial aspirate and tissue aspirate were positive for GBS infection.

Discussion

GBS is a common microorganism, often colonising the gastrointestinal and reproductive tract ³. Rarely, GBS colonises the skin and can cause necrotising fasciitis, i.e. necrotising soft tissue infection (NSTI), with only 22 cases having been reported in the past ii. Majority of these patients are either immunocompromised or have other predisposing factors including recent thoracic intervention or trauma ^{4, 5}.

GBS-related infections of cardiac structures are rare, as a whole, with 2 to 3% of cases presenting as native valve endocarditis and far less as pericarditis, mycotic aneurysms and intraventricular abscesses ³. Parikh et al reviewed the types of microorganisms isolated from purulent pericarditis samples and revealed that only 5% were due to streptococcal organism, sans Streptococcus Pneumoniae, possibly less so due to GBS ⁶. Our literature search revealed only one case of GBS-related purulent pericarditis reported although the case was not linked in any way to a NSTI to our knowledge ⁷.

Our case was unique as, at the time of writing, there were no other reports of GBS-related lower limb NSTI in combination with mediastinal involvement. There have been only a handful of cases of necrotising fasciitis reported with mediastinal involvement, the majority of which were supra-diaphragmatic with only one reporting NSTI of the lower limb due to Aspergillus infection ^{8 9}.

The similarity in culture results obtained from blood, tissue aspirate and the pericardial fluid in our patient suggest dissemination of GBS from the NSTI, possibly via a haematogenous route, although bacterial-related pericardial dissemination can also occur via direct spread from infected foci from neighbouring intra-thoracic structures or sub-diaphragmatic ⁴. The possibility of multi-routed spread should remind clinicians that, albeit rare, mediastinal involvement in NSTI is a possible complication of such disease.

Conclusion

This case highlights the rare possibility of cardiac involvement in cases of NSTI, and the possibility of cardiac tamponade causing cardiogenic shock masquerading alongside septic shock. It also highlights the importance of combining clinical findings with ancillary testing, including bedside echocardiography, when faced with challenging cases of sepsis to help look for possible foci of infection.

Introduction

There have been continuing initiatives to transform and improve the National Health Service (NHS) in recent years. Mental health services in England have similarly shown evolution with regards to service provision. There has been a shift away from the perceived “medicalisation” of treatment, with traditional long-stay institutions replaced with more targeted and personalised care in the community.¹ Furthermore, community services themselves have seen much remodelling over the years including decommissioning and integration, as well as increased involvement in outreach and early intervention teams.²

Mental health services are sometimes perceived as relatively well funded from outside but, as with most healthcare sectors, compared to the population requiring this service, these resources are inadequate to support the growing demand. This has been the case for some time, but it has become more evident with a significant reduction in funding observed since 2010/11.¹ In addition, constant governmental pressures to meet key performance targets, as well as unachievable expectations from the public, have further stretched an already resource-depleted mental health service.

The implementation of new National policies³ was supposed to be a shift from large psychiatric hospitals to smaller specialist community centres with a promised reduction in the demand placed on inpatient services. In England, a peak number of 150,000 inpatient psychiatric beds was reported in 1955; this has since rapidly declined to 22,300 in 2012. Between 2010/11 and 2013/14, a further rapid reduction of 7% of all beds available was seen.⁴

Despite the promise of changes in service delivery within mental health to mitigate the continued reduction in the number of inpatient beds, demand for inpatient beds has not in fact reduced nationally.¹ The recommended level of occupancy, for example, is 85% but 119 wards surveyed⁵ were operating at 91%, with some at 138% level of occupancy. The occupancy levels of over 100% usually occurred when long-stay inpatients were discharged home on short-term leave and their beds got filled during their absence.⁴ Where numbers of inpatient beds fail to meet the demands, or waiting list for their first assessment or review grows, the inadequacy lends these facilities to issues with regard to providing high quality and safe patient care. Examples of this may include inappropriate use of the Mental Health Act for detention of patients as a means of securing an inpatient bed,⁵ incomplete assessments of people detained in places of safety due to time or space constraints,⁶ and an increase in violent incidents on overcrowded inpatient wards.⁷

What is a Crisis Resolution and Home Treatment Team (CRHTT)?

In the late 1980s and 1990s, community mental health teams provided acute crisis support. This posed a number of issues including that these teams usually operated during normal working hours of 9am-5pm (Monday to Friday) and were not always available to provide support to patients in a crisis, and did not have the desired impact of reducing the number of acute admissions.⁸ This gap in service provision inspired the experimentation with and subsequent development of intensive home treatment services, some of which showed evidence of reduced hospital admissions, and holistic-working often preferred by families who were happy to have their loved ones receive the required support in the home environment.⁹ Over the last two decades, with remodelling of services, increased investment, NHS funding rising from £49 billion in 2000 to £122 billion in 2016, and a migration of mental health professionals, CRHTTs were established and are now available in every mental health trust across the United Kingdom (UK).¹⁰

CRHTT is a team of mental health professionals including psychiatrists, community psychiatric nurses, social workers and support workers, who provide rapid and intensive support at home during a mental health crisis.¹¹ They are a 24-hour service operating seven days a week, and acting as the “gatekeeper” for acute services accepting referrals from various sources including inpatient, community, liaison and from outside the Trust for providing support to patients experiencing crises. These teams risk-assess patients and determine whether they require inpatient or home treatment. In the latter case, CRHTTs provide intensive home treatment by offering up to 2-3 visits a day as well as 24/7 phone support. These teams are also involved in facilitating early discharges from hospitals; in cases where patients are past the initial acute crisis, but may need further input prior to discharge to community mental health teams for longer term support.⁸

Definition of diagnosis and second opinion.

A second opinion is defined as “advice from a second expert (such as a doctor/psychiatrist) to make sure advice from the first such expert is correct” whilst diagnosis is defined as “the art or act of identifying a disease from its signs and symptoms”.¹² Due to increased pressure on inpatient facilities and remodelling of community services, there has been a huge increase in the number of referrals made to CRHTTs. Between 2011/12 and 2013/14, it has been noted that referrals to CRHTTs increased by 16%.¹³ Reduction in inpatient beds and high workloads within community services often result in the formulation of arbitrary diagnoses and treatment plans. With increased pressures on other mental health services, the role of CRHTTs has begun to evolve. In addition to the previously discussed functions, CRHTTs appear to be becoming second opinion services by default enabled by the psychiatrists working in these teams.

We organised a project to establish whether a typical CRHTT is fulfilling the criteria of being a diagnostic or second opinion service provider.

Method

We examined 100 consecutively accepted referrals to a CRHTT from 1^st December 2016. The patients were divided into three groups: those being discharged/referred from hospital (HR), those referred from the community (CR), and those who were not open to secondary mental health services at the time of referral (NR). The age range and gender of the groups were noted. Thereafter, the NR group was excluded from analysis for the obvious reason that the CRHTT was not providing a second opinion in their case. The HR and CR groups were further reduced by excluding patients who were not seen by a CRHTT psychiatrist. The remaining patients in both groups were scrutinised regarding a change in medication; this was also recorded for the previous and next care occasions. The likelihood of medication change at the next treatment event was analysed to establish whether it was affected by the previous event. The numbers of patients with CRHTT diagnosis change were also recorded for both groups.

Results

Figure 1: Project Flowchart

Figure 2: Group Demographics

There was little difference in age between the three groups (average ages were: CR=37.8, HR=39.0, NR=36.0). There was a lower proportion of men in the CR group than were present in the HR and NR groups (36% as against 48% and 47%). Whether a psychiatrist saw a patient appeared to be related to both the referral source and the length of CRHTT stay. Most (n=16, 76%) patients in the hospital-referred group (HR) were not seen by a psychiatrist while most (n=24, 67%) of those referred from the community (CR) did receive such an outcome. No community-referred patient was seen by a psychiatrist if they were with the CRHTT for less than a week. These short-stay patients accounted for 7 out of the 12 community-referred patients who were not seen. This suggests that a psychiatric assessment should be scheduled more quickly after community referrals so as to offer patients a more comprehensive service.

Psychiatric assessment led to changed diagnoses for 28% (8/29) of patients. This figure was 40% (2/5) for the HR group and 25% (6/24) for the CR group.

Medications were changed for 69% (20/29) of patients seen by a psychiatrist. In the subgroups; 60% (3/5) of HR psychiatric assessments resulted in a change of medication while 71% (17/24) of CR psychiatric assessments led to medication changes.

The chi-square statistic was used to evaluate whether a recent medication change, during the inpatient stay or at the most recent outpatient appointment, made the CRHTT less likely to adjust medication. This indicated that there was no relationship between the two events. A similar analysis indicated that the likelihood of a medication change at the patient’s next community appointment was increased by seeing a CRHTT psychiatrist but unrelated to whether that assessment had resulted in a change of medication.

Discussion

We have demonstrated in this study that a typical CRHTT is providing a diagnostic and second opinion service. Changes in medication were more than twice as frequent as changes in diagnosis – this is perhaps unsurprising as diagnostic changes would be likely to require a different prescription.

Most community referrals were actively evaluated in terms of both diagnosis and treatment. This is a significant change to the original function of the CRHTT where a psychiatric assessment was not a standard aspect of care when very few of the original CRHTTs included a psychiatrist. This may also reflect the current pressures on community teams, which are frequently short-staffed, leading to more competition for the available clinic appointments. Consequently, patients may not have seen a psychiatrist for some time and their requirements may have changed. It is, however, also known¹ that community patients who have not been reviewed recently or who have a long wait before their first assessment are more likely to present in crisis.

The diagnostic and second opinion function of the CRHTT is more prevalent when patients have been referred by the community team (67% reviewed, 47% medication changed) rather than on discharge from hospital (24% reviewed, 14% medication changed). This appears to largely reflect the fact that relatively few discharges were seen by the CRHTT psychiatrist because these patients had just received a full consultant-led discharge treatment plan. This may be another example of community service pressures leading to patient crises and thus engagement with alternative services – in this case inpatient care may be offering a second opinion service. The current separation of community and inpatient services will augment this effect as previously the patient would have been more likely to receive continuous care from the same consultant. This is an interesting view of current service configuration. The reduced continuity of care is often seen as a disadvantage but it does present an opportunity for a fresh evaluation of a patient’s diagnosis and medication by a different psychiatrist.

Longer lengths of stay with the CRHTT made psychiatric assessments more likely. It was particularly clear that discharge within a week made a psychiatric review unlikely. The proportion of community-referrals seen by a CRHTT psychiatrist could be increased to 83% if patients were to be seen within 24 hours. This figure is derived from the assumption that psychiatrists would then see the same proportion of both long and short stay patients. The residue would include those patients who refuse to engage with such an appointment.

It is interesting that chi-square statistical analysis suggests that the only influence on prescription change at the next appointment is whether the patient was seen by a CRHTT psychiatrist. It is not related to whether or not the CRHTT psychiatrist changed the medication. It is difficult to see why this should be the case unless the community psychiatrists consider the patients’ needs in more detail or are tempted to regain control after the referral to another psychiatrist.

In conclusion, the addition of psychiatric care to CRHTTs may be a valuable adjunct to the current pressures on community teams. The current trend to separate community, inpatient and CRHTT care is often cited as a disadvantage due to reduced continuity of care for patients. This project has drawn attention to the fact that it also offers opportunities for new teams to re-evaluate both diagnosis and treatment which offers patients the advantage of an internal second-opinion service. This advantage could be offered to more community-referred patients, albeit with more resources, by ensuring that they are assessed by the CRHTT psychiatrist within 24 hours.

Limitations

This is a small study conducted in a single CRHTT. It does, however, offer an indication of the evolving role of the CRHTT and its relationship to other services.

My countryside

home is where the heart is

at its gladdest and lightest,

like a hot air balloon

floating gently over

emerald green fields

and golden dirt tracks.

My rustic

home is where the heart is

far from health services,

far from those specialists –

like cardiologists –

who can stop diseases in

their concrete-covered tracks.

My bucolic

home is where the heart is

more likely to fail –

liable to stop beating

earlier than all those

hearts that thump out their

rhythms in the city.

My rural

home is where the heart is

woven into the patterned fabric

of a vibrant community,

whose median age

keeps rising and rising

as fond memories fade.

My countryside

home is where the heart is

destined to be

till its last beat.

Background and rationale

Psychiatric trainees in Iraq face many challenges that limit their immediate access to improved training opportunities. These include limited access to classroom teaching, regular clinical supervision meetings and fewer opportunities to attend international conferences and placements. These challenges are more acute in those specialities with the greatest shortage of consultants (for example, forensic and child and adolescent psychiatry).

Furthermore, the fragile security situation in the capital and larger cities and the post-conflict disruption to educational institutions consequent to these difficulties makes it difficult for those in the UK and elsewhere to visit the country and support educators and training on the ground.

Against this background and as a medical educational team in the UK (Oxford University Medical Education Fellows, OUMEF) with an interest in developing training opportunities for peers and colleagues in Iraq, we set up the Oxford Psychiatry in Iraq (OxPIQ) Project, beginning with a project development team that included Medicine Africa, an experienced online distance learning platform.

So what is the role of TEL in the delivery of online distance learning targeted at medical professionals in these circumstances?

Meeting the Challenge – the role of TEL

The concept of Technology-enhanced Learning (TEL), or Web-based learning (WBL), defined as the use of information and communication technologies in teaching and learning ¹, is a relatively new phenomenon. Nevertheless, there is a considerable body of evidence supporting the use of TEL in various clinical and non-clinical settings.

Mccutcheon et al. ² systematically reviewed thirteen studies and found that ten of these studies concluded that online learning is as effective as traditional or classroom teaching, despite the limitations of some of these studies.

In a large meta-analysis, Means and colleagues ³ concluded that students using online teaching performed modestly better compared to students learning similar material using face-to-face teaching. Combining face-to-face and online teaching resulted in larger benefit compared to the use of face to face methods only.

TEL can address the learning limitations in classroom settings due to expanding curriculum coverage and limits on contact time between students and lecturers/trainers alike. It can contribute to better use of such face-to-face classroom contact through the facilitation of the flipped classroom approach. ⁴ In this approach (also called inverted instruction and upside-down teaching), students acquire the basic information of the lesson outside the class (usually using online materials) and then develop their knowledge further in the class by sharing their learning, interacting with other classmates and teacher, and discussing various aspects of the study topic.These advantages have enabled TEL to revolutionise distance learning at many levels – enabling greater access to education by overcoming geographical and time-zone boundaries.

An allied concept within distance TEL is the concept of virtual teams ⁵ where health professionals come together to teach and learn from each other independent of location. Of itself, this offers some advantages. These include the possibility of addressing speciality-specific training gaps through the incorporation of the relevant expertise within the team - and to the creation of what is termed “connectivism”. This term refers to the use of internet technologies to enhance learning through online peer networks ⁶ and the development of communities of practice. ⁷ The latter allows for workplace-based learning with trainees learning from more experienced practitioners and moving towards the same through greater competency acquisition.

In a similar vein, creating networks of professionals may help to establish more longer-lasting relationships of mutual benefit between the UK and Iraqi professionals (e.g. through collaboration on training programmes, conferences, etc.). Also, cross-cultural online learning has been shown to be very useful in improving language skills and cultural awareness of learners and educators. ⁸ With language translation technology, any language difficulties can also be overcome, especially if the educator can observe the learners’ responses to the translated text and offered the opportunity to give further explanations and clarifications when necessary. ⁹ Finally, as well as sharing knowledge and experience within groups, TEL enables opportunities for mentoring and coaching individuals. ¹⁰

For our purposes, these findings and opinions support the use of online learning as a suitable distance learning “add-on” to existing training opportunities in Iraq.

TEL and Learning Theories

Learning theorists suggest that experiential and constructive learning theories are most appropriate to learning in the clinical context. Both are possible with TEL (as well as being facilitative of behaviourist and cognitivist approaches).

For example, the virtual classroom environment can enhance the learning experience of the participants by improving their analytical skills by thinking through case formulation and management plans. ¹¹ Participants in online learning could be assessed and receive the feedback immediately. Ideas can be shared, and there is no passive acquisition or transfer of knowledge as is the case with traditional lectures. These aspects have implications for the design of the educational sessions and are discussed below in the learning methods section.

Challenges of Online Distance Learning

There are many challenges associated with online distance learning. Firstly, there is the potential lack of the required technologies (internet access, laptops or desktop computers), the expenses of subscribing to these online learning templates, the need to have technical support, and similar technical and logistic issues. ¹² These technical problems may impair access to and functioning of the virtual team. The choice of an experienced online platform must, therefore, be considered carefully.

Secondly, there may be ethical issues about the protection of patients’ confidentiality in these sessions, especially when there are different laws of privacy that are applied in the UK and Iraq. This will require knowledge of the relevant professional requirements by the tutor team for example.

Furthermore, the student-teacher relationship has traditionally been underpinned by direct face-to-face contact and being present at the same time and place. ¹¹ Therefore, learners and educators might be less satisfied with online learning. For these reasons, the concept of blended learning (careful integration of online learning with face to face learning experience) has been developed to overcome the limitations of a standalone online or face to face learning and has been found effective and applicable in various settings. ¹³

Thirdly, any distance online learning programme must understand and support existing “local” training provision and arrangements, in the classroom and the workplace. This requires liaison and cooperation with the training providers and institutions on the ground.

For clinical training to be relevant, it needs to reflect the learning needs of trainees in the workplace – in keeping with adult learning principles and cognitive apprenticeship models of learning. ¹⁴ The latter includes the importance of clinical decision-making underscored by the higher levels of Bloom’s (1956) cognitive domain. ¹⁵ To this end, then appropriate learning and assessment methods are needed to enable effecting learning.

In other words, while necessary, TEL may be insufficient in enhancing learning outcomes if allied learning methods are not chosen appropriately. Also, in our view, TEL is not a substitute for bedside teaching.

Table 1 summarises this appraisal of online distance learning (using the online platform provided by MedicineAfrica).

Table 1 Strengths and limitation of using MedicineAfrica (web-based virtual classroom environment)

OxPIQ & Project Development Team

OxPIQ is a partnership between Medicine Africa and psychiatrist members of the Oxford University Medical Education Fellows, with experience of working in Iraq. The Oxford University Medical Education Fellows (http://OUMEF.org) is a group of trainees from across medical and surgical specialities with interest in medical education and training.

Medicine Africa (http://medicineafrica.com) is an innovative clinically targeted online platform developed in collaboration with King’s College London’s Centre for Global Health, within the King’s Somaliland Partnership. Built at low bandwidth, it enables collaboration between medical professionals in the UK and those in remote or fragile states to enhance education in various clinical specialities using online sessions (live courses and mentoring sessions). Please see Appendix 3 for a screenshot of one of the active sessions of OxPIQ.

Following the development of a project team, additional team members were added from the Subcommittee on Iraq of the Royal College of Psychiatrists (http://www.rcpsych.ac.uk/workinpsychiatry/internationalaffairsunit/iraqs...), thus completing the UK dimension.

The next step was to invite representation and support from the Iraqi Board of Psychiatry and the Medical Education Unit in Baghdad. These developments led to the formal launch of OxPIQ Partnership in March 2016. Later on, the many UK and Iraqi doctors joined the Partnership as tutors and learners.

The Virtual Learning Team: Trainees, Specialty Consultants & Tutors

Iraqi psychiatry trainees were then recruited, and their more pressing learning needs to be appraised based on their views and those of the Iraqi Board of Psychiatry supervisors. Learning needs to emerge included the management of older patients with dementia and functional disorders, assessment and management of children and adolescents (with autism and ADHD for example), forensic patients and those with drug and alcohol addiction. The team thus formed was composed of up to ten psychiatry trainees from Iraq and five senior psychiatrists/tutors each, from Iraq and the UK respectively. A schedule of fortnightly seminars was agreed and published on the learning platform. Case-based discussions were used as the main educational activity during these seminars.

Learning Methods and Processes

As noted earlier, the importance of experiential and constructivist learning methods are key to clinical education. Our literature appraisal revealed that they are essential elements of successful TEL in this context too. ^{16, 17} To these must be added learner engagement. ¹⁸

Virtual or online (anonymised) case-based discussions (CBDs) are valid and reliable learning tools. ¹⁶ They are interactive and centred around the students and their learning needs while a facilitator guides the process of learning. Learners are engaged through discussion of actual clinical cases, so preparing learners for real-life experience. ¹⁹ Also, expert facilitation and peer feedback to trainees promotes clinical knowledge and skills’ development. ^{20, 21}

Effective small group teaching is characterised by four main strengths: flexibility, interaction, reflexivity and engagement. ²² Flexibility is when the teacher responds to the needs and learning of the students dynamically and helps them to explore wider pedagogic spaces. A higher degree of interactivity is usually seen in small group teaching compared to a larger group. Teachers are better able to continually engage in self-reflection and listen sensitively to students in a small group and observe the dynamics between the members of the group, leading therefore to better reflexivity. Engagement refers to encouraging the students to develop their academic identity and engage in lively debate about the various aspects of the topic discussed.

We aimed to replicate these characteristics. For example, a small group discussion allowed better interaction with each participant (interactivity); the presence of chat windows enables the facilitator to self-reflect on the process, monitor engagement and respond reflexively using questions and answers to stimulate interest and respond flexibly to individual trainee knowledge gaps. Tutors are encouraged to identify trainees’ learning needs and facilitate interactivity, and timely feedback as these are highly valued by the participants and help to keep them motivated and engaged. ¹⁸

For further reading in this area, we recommend Brindly and colleagues’ ²³ ten strategies to increase students’ motivation towards and engagement with online learning (see table 2).

The focus of the Lesson Plan Design

To these ends, the focus on the lesson design was on using problem-based learning methods (e.g. CBDs) within a small group setting (between 4-12 members) and a format that promoted learner engagement. A sample lesson plan is provided in Appendix 1.

In practical terms, tutorials were held fortnightly in term-time. All participants received an email notification to inform them of the session topic, and the tutor uploaded the slides from the session to the website beforehand. Participants logged-in to the site (http://medicineafrica.com) and interact with the tutor by voice (requiring only simple microphone equipment) and by writing in a chat window.

Evaluation and feedback gathering

The evaluation of the effectiveness of these sessions was reliant originally on trainees’ immediate reaction (table 3, level 1 evaluation, Kirkpatrick ²⁴) using formal feedback tools provided online by MedicineAfrica. This feedback was shared with tutors and the Project Team. Please see Appendix 2 for the template used in collecting feedback after each session.

Subsequently, members of the project team approached trainee representatives, tutors and Iraqi Psychiatry Board leads separately for further feedback and appraisal of learning needs. Furthermore, some months after a tutorial we have asked trainees for evidence of learning across the higher levels of Kirkpatrick’s evaluation model.

Regular feedback from the Iraqi and UK participants has been positive. The sessions have been associated with improved clinical knowledge and skills of the Iraqi Psychiatry Trainees. Requests for certificates of tutorial participation have been agreed upon and provided by the project team addition, so supporting learners’ (and tutors) portfolio development.

Table 3 Kirkpatrick’s (1996) Levels of Training Assessment

Further cooperation

A surprising (and very welcome) outcome of the project was, through the facilitation and support of the Iraqi Board of Psychiatry, the introduction of educational workshops in Baghdad. These workshops were held in Medical City, Baghdad, in May 2017 and April 2018 and were facilitated by tutors (YH & H Al-T) from the OxPIQ Partnership. They covered targeted topics such as old age psychiatry, addiction, organic and forensic psychiatry. Trainees and senior psychiatrists from Iraq attended; their feedback showed how they valued the interactive nature of the teaching and use of CBDs as learning methods, resulting in high levels of engagement.

Conclusions

This paper describes the process of designing, delivering, and the early evaluation of an online distance TEL programme for mental health professionals based in the UK and Iraq.

TEL has had an important role in overcoming the geographical barriers and other challenges to developing training opportunities in Iraq and other developing countries. We are of the view that it could be used more often to connect professionals working in similar circumstances and with other disadvantaged groups, including refugee and asylum seekers. It is a flexible way of providing training to professionals working with those groups in relatively remote and resource-deprived environments.

Greenhalgh ²⁵ suggests that three factors are needed for the success of online educational activity: ease of access, perceived usefulness of the activity to the learning requirements of the students, and the interactivity of the session. In our experience, these are important. Also, we believe that additional consideration should be given to (i) working with an experienced online platform provider; (ii) working with local educational institutions, trainers and learners to identify unmet learning needs and support existing learning opportunities/programmes; and (iii) adopting an iterative approach to feedback and evaluation.

Appendix 1: Example of a Lesson Plan

Appendix 2: Feedback form to be completed by the participants after the session

Appendix 3: MedicineAfrica screenshot during an active session

The recent increase in the number of patients presenting with a borderline personality disorder (BPD) in general adult psychiatry and primary care is creating pressure within the National Health Service (NHS)¹.Currently, BPD is perceived to be like an ‘epidemic’ entity, particularly in areas with a high incidence of socioeconomic deprivation. Similarly, there is a parallel increase in the human and medical resources needed to manage this disorder efficiently. In fact, the authors have found that BPD tends to be comorbid with factitious disorders and depression (Tripolar syndrome) with a tendency to overuse hospital and medical facilities, inclusive of Accident and Emergency (A&E) departments, family doctors and General Practitioner (GP) surgeries².

Consequently, patients with BPD require a constant and unlimited allocation of medical and psychiatric resources, together with targeted care plans. In fact, they might be prone to frequent self-referrals to A&E, seek hospital admissions and augment all their psychotropic medications in order to deal with their on-going crises not solvable in their homes. Also, the skills needed by healthcare personnel to reduce chronic self-harming and suicidal ideation in this vulnerable population are complex and need to be updated on an on-going basis also due to the tendency of these patients to raise allegations towards their healthcarers³. Nonetheless, the provision of treatment is often hindered by various healthcare system limitations, such as the lack of beds on medical and psychiatric units, forced reduction in the length of stay in a hospital and insufficient human resources. This scenario has mostly affected female patients with BPD who seek admission to psychiatric hospitals often for respite from chronic suicidal ideation⁴.Moments of amplified suicidal ideas become evident when internal voices, perceived as auditory hallucinations commanding to self-harm or to commit suicide, become more intense⁵.

As observed by the authors of the current editorial, increased suicidal ideation in persons with BPD also occurs during minor crises in life, when experiencing intensified flashbacks about past abuses, during minor losses, after significant conflicts with others and after the separation from influential people in their social network. Besides, admissions in psychiatric wards, very commonly, occur when there is an intensification of internal voices commanding BPD patients to take overdoses of the prescribed medication or to jump in front of a train, a car or off a pier to commit suicide. Police is often involved to stop these dramatic plans. At the same time, healthcare professionals are discouraged by the complex management of patients with BPD, which, in combination with their tendency to challenge or make unwarranted allegations against their health carers, results in feelings of sadness, rejection and alarm in the latter. Kanin reported that the reason to produce a false allegation is to create a defence or to get compassion⁶. Nonetheless, it is also likely that some healthcare professionals might have some preconceived ideas about people with Borderline Personality Disorder, which might reduce the depth of health carers’ empathy towards these patients and lead to burnout after prolonged treatment of BPD in hospital or community. Attempts to treat and to reduce suicidal ideation and self-harm in this group of patients are often thwarted as they challenge medical decisions and endeavour to sabotage the proposed care plans. The strain on the doctor-patient relationship is determined by the underlying ‘Mistrust/Abuse’ scheme of patients with BPD who expect from others, and are thus sensitive to, signals of relational wound, treachery and abuse⁷.

Consequently, a chronic feeling of inadequacy in patients with BPD translates itself in enduring dissatisfaction with any therapy and healthcare professionals. Hence, in the authors’ experience, any attempt to establish a long-term therapeutic relationship with BPD patients might have limited outcomes. Frustration in healthcare professionals aiming to create an enduring therapeutic alliance with patients with BPD happens as these patients tend to interpersonal biases and to ascribe undesirable experiences to people (hence to healthcare professionals) as opposed to circumstances⁸. Therefore, social interactions with primary carers result in dissatisfaction of people with BPD about any medical or psychiatric plan is set up for them. Consequently, community teams, general practitioners and hospital staff feel hopeless due to recurrent readmissions of people with BPD and the lack of definitive treatment for such pathology. Stress caused by difficulties encountered in ensuring that BPD patients comply with the therapy regularly places doctors and nurses at crisis point.

Once in the hospital, discharging patients with BPD can be difficult as they are frequently reluctant to return to the community, leading to recurrent readmissions within a short period. In fact, the period before discharge from a psychiatric hospital is complicated by mounting anxiety and distress in patients with BPD. The authors observed a regular escalation of self-harming behaviours and increased suicidal ideation in these patients just before discharge, possibly indicating their underlying anxiety in going back to the home environment. Many BPD patients suggest that they would rather stay in the hospital instead of returning to the community that is considered by them as unsafe or unstructured. Furthermore, as these patients have an intense vulnerability to social rejection, they rarely feel adequate during social interactions thus developing an enduring sense of solitude⁹. Therefore, any hospital discharge or a visit to the GP will be interpreted by them as disappointing and will lead patients with BPD to confirm their sense of rejection. As a reaction, the authors observed that BPD patients demand endless and unconditional attention from their primary carers. Attempts by patients with BPD to self-harm or commit suicide intensify over weekends or public holidays as their sense of solitude increases during these periods, especially when there is also a shortage of healthcare professionals available for immediate support.

The authors of the current editorial propose possible strategies of intervention both on the psychopharmacological and managerial side. The challenge is that patients with BPD often use overdoses of oral medication in a suicide attempt¹⁰. Hence, the authors recommend the use of long-lasting depot antipsychotic injections (e.g., Zuclopenthixol Decanoate) to stabilise their mood and reduce impulsivity, the risk of overdoses, pseudo-psychotic symptoms and command hallucinations leading to deliberate self-harm. The use of oral lithium to treat mood swings poses an ethical dilemma for doctors as it could be lethal when used as an overdose. Healthcare management is another way of intervention. One point of difficulty is the tendency of patients with BPD to split their teams and to create niches of protectors and opposers within staff with possible conflicts within the team that is treating them. In this case, inter-professional coordination, integrated care and constant information sharing are required¹¹. Furthermore, several healthcare services treating patients with BPD are trying to find an integrated approach for their hospital and community treatment. The authors speculate that the increased number of admissions of patients with BPD is reducing the total capabilities of physical and mental wards to treat patients with other pathologies. Besides, the dramatic presentation of patients with BPD who tend to overuse the healthcare services poses ethical dilemmas in their management. This scenario has created discrepancies in health care policies about treatments and hospital (re)admissions of patients with BPD reaching an epidemic magnitude in many healthcare trusts. Hence, a new culture is required for the management and treatment of patients with BPD in the community.

Culture is defined as the character of an institution that affects employee gratification and organisational accomplishments¹².What is needed is a frank and constructive dialogue between healthcare managers, leaders and medical staff in the hospital and in the community. Furthermore, clear and regional guidelines should exist to improve the efficacy of care which is offered to BPD patients at home and to reduce the constant risks which patients pose to themselves, their sense of solitude and their tendency to seek hospital admission in order to solve chronic existential difficulties. A model of integrated care comes from Max Weber who differentiated between ‘formal rationality’, the endorsement by healthcare managers of the most efficient ways of achieving organisational goals (e.g., ensuring more hospital beds by quick discharges of ‘bed blockers’), and ‘substantive rationality’, the expectation by healthcare professionals that values and morals should instead be based on tradition, compassion and dedication¹³;pertinent to the care of BPD patients in our case. The collaboration of all those involved parties is also important to reduce the risk of ‘silo management’ where confined and regional policies do not embrace a wider perspective for the management of specific problems while responding only within the confines of the own guidelines and procedures¹⁴.In these cases, integrated care in communities can halt self-harming and suicidal attempts of patients with BPD. The organigram sees inter-professional actions, targeted psychopharmacological policies and psychiatric crisis teams in A&E that can reduce the need to hospitalise patients with BPD at any ensuing crisis.

A dermatoscope is a hand-held device for examining the appearance of the skin. Dermoscopy has become an increasingly used and valued tool in the assessment of various skin lesions, and more recently, inflammatory rashes. It is quick, cheap and when used correctly, dermoscopy is an essential tool in helping clinicians detect early stage skin cancer. Various national and international guidelines recommend routine use of dermoscopy in the assessment of pigmented lesions^1,2 because it enhances melanoma detection rates^3,4 and can help confirm the diagnosis of benign lesions such as haemangiomas and seborrhoeic keratoses. As with any skill, competency takes time to develop and a combination of various learning and assessment methods is best. The dermatology specialist training curriculum in the United Kingdom (UK) states that trainees should be competent in using a dermatoscope and interpreting findings, while recognizing the limitations of this tool⁵. Assessment of these clinical skill and behavioural competencies using direct observation of procedural skills (DOPS), case-based discussion (CBD), mini clinical examination (mini-CEX), and/or multisource feedback (MSF) is suggested. There is no specific guidance on what resources a trainee should use to achieve these competencies, nor on what is the minimum expected dermoscopy skillset at completion of specialist training.

The aim of this survey was to explore dermoscopy use amongst dermatology specialist trainee registrars in the UK including frequency of use, how it is being taught and whether trainees feel their dermoscopy training has been adequate.

An online survey was designed and distributed to dermatology trainees in the United Kingdom using an email link and hard copies were distributed at a national dermoscopy course. Respondents who did not identify themselves as dermatology trainees were removed from the analysis. Responses were collected anonymously, then collated and analysed using SurveyMonkey® computer software.

Twenty-five percent (59/238) of dermatology trainees completed the survey. On average, 92% (54/59) use dermoscopy more than once daily. Eighty-five percent (50/59) always use dermoscopy when assessing pigmented lesions while 34% (20/59) always and 59% (35/59) sometimes use it to assess non-pigmented lesions. When asked about specific tools used to learn dermoscopy, 41% (24/59) have been on a previous course, 42% (25/59) reported attendance at a lecture or seminar, 46% (27/59) have used a dermoscopy text book, 14% (8/59) have attended a conference, 19% (11/59) have used online resources. Seventeen percent (10/59) have never used any of the above learning methods. (Figure 1a). Amongst those who have attended a formal dermoscopy course (n=24), 92% (22/24) of these were ≤1 day in duration. When questioned about informal teaching in clinical practice, 12% (7/59) frequently, 56% (33/59) sometimes, 31% (18/59) rarely and 2% (1/59) never receive teaching from their supervising dermatology consultant. (Figure 1b). Fifty-four percent (32/59) feel they have received adequate training in dermoscopy while the remaining 46% (27/59) feel their dermoscopy training is inadequate for their training stage (Figure 1c). Seventy-three percent (43/59) have access to dermoscopic photography within their local dermatology department.

Fig 1a - Have you undertaken any formal study in dermoscopy? 49% of trainees have attended a lecture, 2% a seminar, 14% a conference, 41% a course, 19% have used an online resource, 46% have used a book, 17% have not used any resource.

Fig 1b- Do you receive dermoscopy training from your supervisor in clinic? 56% of trainees sometimes, 31% rarely, 12% frequently, and 2% have never received training from their seniors in clinic.

Fig 1c- Do you believe that you have received adequate training in the use of a dermoscopy for your training grade?

These results of this survey highlight the need for dermoscopy training to be reviewed within the UK national training curriculum for dermatology. Despite daily use by the vast majority, dermoscopy training is largely self-directed and highly variable amongst individual trainees. Of concern, a significant proportion of those who responded feel their dermoscopy skills are inadequate for their training stage. Of note, the 25% response rate means that the results of this survey may not be representative of dermatology trainees in the United Kingdom as a whole.

This is the first time that dermoscopy use has been explored through a national survey of dermatology trainees in the UK, to the best of our knowledge. A survey on dermoscopy use was carried out by The British Association of Dermatologists (BAD) in 2012⁶ but the majority of responses were from dermatology consultants. This confirmed that 98.5% of respondents regularly used dermoscopy, while 81% had received any training. The most frequent source of training was UK based courses, which 62% of respondents reported attending. Of note, 39% of all respondents lacked confidence when making a diagnosis based on their interpretation of dermoscopy findings. It is not clear how many of those lacking in confidence were consultants, trainees or specialty doctors. Although the situation may have improved since 2012, these results do suggest that dermoscopy training needs have not been met for a proportion of doctors across the dermatology community.

Dermoscopy training is an important issue to address for several reasons. The volume of cutaneous lesions being referred to dermatology is increasing, and skin cancer referrals and treatment now account for 50% of a UK dermatologists’ workload⁷. For every melanoma diagnosed, a dermatologist may expect to see 20–40 benign lesions referred from general practitioners (GPs)⁷. These facts highlight the importance of maximising diagnostic skills which frequently include using dermoscopy as part of clinial assessment. Lack of adequate training is a common self-reported reason for dermatologists not using dermoscopy⁸. Both trainees and their supervising bodies have a responsibility to maximize training opportunities and embed the use of dermoscopy in routine practice.

In conclusion, we feel UK dermatology trainees and indeed any clinician who utilizes this tool, would benefit from a more standardized and integrated approach to dermoscopy teaching to ensure safe practice of this skill and deliver high quality evidence-based patient care.

Introduction

With the increasing use of ultrasound as a standard examination in the first trimester, more incidental adnexal masses are detected. The reported incidence of adnexal masses in pregnancy varies, depending on the criteria used to define the mass. A literature review by Goh W. et al., found that 1% of all pregnancies are diagnosed with an adnexal mass ¹. A more recent article has suggested adnexal masses are diagnosed in 5% of all pregnancies ². Simple and functional cysts are very common, and they usually resolve after the first trimester ³. Mature teratomas are by far the most common persistent adnexal masses found in pregnancy ⁸. It has been estimated that up to 5% of adnexal masses in pregnancy are malignant ⁴.

Ovarian cysts are typically asymptomatic, but they can cause pain due to pressure on adjacent organs, rupture, bleeding or torsion. The latter case is a significant health condition which mainly requires emergency surgical intervention. During pregnancy, surgical management of ovarian cyst complications is more difficult and more challenging. This is mainly because of other differential diagnosis causing similar symptoms related to pregnancy such as ectopic pregnancy and miscarriage. In case of surgical intervention, the second trimester of pregnancy is supposed to be the safest window for surgery as the risk for drug-induced teratogenicity is smaller than in the first trimester, most functional cysts have disappeared by then and it is technically less difficult than operating during the third trimester ¹³.

Antenatally, ultrasound is considered to be the best first-line imaging to evaluate adnexal masses ⁵. Ovarian mass characterization into benign, malignant or borderline can be challenging in pregnancy. This is mainly due to the effect of high levels of gestational hormones which can cause decidualisation of the cystic or solid parts of the ovaries. Benign masses can mimic malignant masses due to this pregnancy related phenomena ¹². One of the largest data in literature on ovarian mass characterization is published by the International Ovarian Tumor Analysis group (IOTA). All IOTA studies excluded pregnant women when they developed and validated the rules and models to characterize ovarian masses ^14-17. This limits our knowledge and ability to use these models in pregnant women. It is known that tumour markers may be raised in pregnancy and should therefore not routinely been done ⁷. An alternative diagnostic tool is Magnetic Resonance Imaging (MRI) which is considered to be safe in pregnancy and can be helpful if the ultrasound imaging is inconclusive in evaluating whether a mass is benign or malignant ^{6; 10}. The American College of Gynecology and Obstetrics recommends that pregnant patients should be reviewed on a case-to-case basis and stated that there are no known biological effects of MRI on fetuses. However, Gadolinium, which help in characterizing ovarian masses, should be avoided when examining a pregnant patient ¹¹.

The aim of this retrospective study was to look into characteristics, size and subsequent management of cases of adnexal masses in early pregnancy.

Methods

This was a retrospective study of data collected between 12/01/2014 and 14/11/2016 in the Early Pregnancy and Gynaecology Unit (EPAGU) of a tertiary referral centre (Guy’s and St Thomas’ NHS Trust, GSTT) in central London. The Ultrasound reporting system (Astraia Software Gmbh, Version 1.24.10, Munich, Germany, 2016) was searched for data. Cases included were consecutive. The study was approved as a service evaluation audit by the Clinical Governance team at Guy’s and St Thomas’ NHS Trust. The study included women who were diagnosed with an adnexal mass while having a transvaginal ultrasound scan TVS at or before 15 weeks of gestation. Pregnancy was confirmed by a positive pregnancy test and an intrauterine gestation on transvaginal ultrasound scan. Women who had the first gestational TVS after 15 weeks of gestation, pregnancies of unknown location, ectopic or trophoblastic pregnancies and patients who had ovarian stimulation treatment were all excluded.

Repeat ultrasound scan reports were retrieved from the Astraia system. Further procedures, tests and imaging results were retrieved using the Electronic Patient Reporting system at GSTT (EPR application, iSOFT Group plc., USA, 2004), PACS (GE Medical Systems, Wisconsin, USA, 2006), Badgernet (Clevermed, Client version 2.9.1.0, Edinburgh, UK). We have used the subjective impression of the examiner as the index test. If surgery was performed the final outcome to identify benignity or malignancy was considered to be the histological diagnosis if any removed tissue. Cytology was used for a reference test in two cases when ovarian cysts were aspirated only. Borderline tumours were classified as malignant for statistical analysis. Tumours were classified using the criteria recommended by the World Health Organisation (WHO) ^{9; 10}. All ultrasound scan images were available and reviewed by author TEG to confirm the US finding. For statistical analysis, the SPSS software package was used (version 24 for Windows, Chicago, IL, USA). A two tailed student’s t test was used to compare means in ovarian masses diameters and a p value of less than 0.05 was considered statistically significant.

Results

7150 patients underwent transvaginal scans for early pregnancy in that period. In total 48 cases of women with adnexal masses in pregnancy and completed data were analysed. Seven women have been excluded; one woman being postpartum at the time of the finding of a large endometrioma, five pregnancies due to assisted conception and one woman was found to have a corpus luteum cyst (Figure 1).

Figure 1: Study flow chart.

41 women with 46 ovarian cysts could be included in the study. Two women had bilateral ovarian cysts, one had two ipsilateral cysts and one woman had three ipsilateral cysts. The mean age at the time of detection of the ovarian mass was 30 (95%CI:28-32) (Fig.2).

Figure 2: Age distribution in the study group.

The mean gestation at the time of first ultrasound was 7.4 weeks (95%CI:6.6-8.3). The mean diameter of ovarian cysts measured 47.7mm (95%CI:39.9-55.4). In 36 women ultrasound alone was performed to reach diagnosis, one woman had an extra MRI scan, two women had tumour markers on top of the TVUS and in two women an MRI scan and tumour markers were performed after the TVUS. The ovarian cyst(s) was on the right ovary in 16/41 women, on the left in 22/41, bilateral in 2/41 and in one case the side of the cyst was not reported. The most common ultrasound subjective impression was mature teratoma (22/46 cysts), followed by simple cysts (12/46 cysts), haemorrhagic cysts (6/46 cysts), endometriomas (5/46 cysts) and one possible mucinous Borderline tumour. The latter was confirmed later on histology as the stage FIGO 1A intestinal type mucinous Borderline tumour (Fig.3).

Figure 3: Distribution of origin of cysts by histology.

In total 8/41 women (19.5%) underwent surgical intervention; of these eight cases six underwent major surgery under GA and two had a cyst aspiration under local anaesthesia. Seven out of these eight masses were classified as benign on USS and were subsequently confirmed to be benign by histology or cytology. In only one case a complex adnexal mass was found on USS examination at 9 weeks of gestation and the MRI scan reported possible malignancy. Tumour markers in this 23-year-old woman were normal and a laparotomy was performed at 17 weeks of gestation to remove the mass. Histology showed the mass to be a mucinous borderline tumour, FIGO stage IA. In another patient, an oophorectomy had to be performed at the time of the Caesarean section at term for fetal distress as the ovary was found to be necrotic. In this patient an ultrasound at 10 weeks of pregnancy had demonstrated a 6cm diameter haemorrhagic cyst, which had presumably torted during the pregnancy without any symptoms to prompt the patient to refer herself. Histology in this case had shown an infarcted cyst with fibrosis and calcification. In four of the major surgery cases performed under GA uncomplicated laparoscopies were performed to remove the adnexal mass; in one case a laparoscopic salpingoophorectomy was performed as an emergency for a suspected ovarian torsion at 16/40 weeks. In three cases a laparoscopic procedure for cystectomy was performed electively for ongoing pain. In the first case a cyst was diagnosed in early pregnancy subsequently there was a miscarriage and the cyst was removed 4 months after the diagnosis. In the second case a cyst was found in early pregnancy the woman had a termination at 11 weeks of pregnancy and a cystectomy 5 months later. In the third case a laparoscopic cystectomy was performed 8 weeks after the diagnosis, however the woman suffered a miscarriage at 12 weeks of gestation. Histology confirmed dermoid cysts in all four of these cases. The two cyst aspirations performed under local anaesthesia and ultrasound guidance were both symptomatic for torsion, one at nine weeks and one at ten weeks of pregnancy. In both patients the procedure has been successful. 33/41 (80.5%) with no indication for surgical intervention. There was a significant difference in the mean diameter of ovarian cysts in the expectant management group (41.2mm; 95%CI:34.7-47.7) compared with the mean diameter of cysts in the surgically managed group (74.5mm; 95%CI: 49.2-99.8) (Fig.4).

Figure 4: Mean diameter of the ovarian cysts.

In 33/41 patients no surgical intervention was needed during pregnancy. 13/33 patients had no follow up of their ovarian cyst arranged and no further mentioning of the cysts on routine growth and anomaly scans during pregnancy was found. In 20/33 patients at least one routine follow-up scan was performed 1-2 weeks after the diagnosis and in 12 of these 20 patients a second follow-up had taken place at least 1 month after the diagnosis. In one of the 20 patients with recorded follow-up’s an MRI scan had been arranged 2 months after the initial USS finding of a dermoid cyst.

Discussion

The results of our study confirm findings from previous studies: The vast majority of ovarian masses in pregnancy are benign and invasive cancer in pregnancy is rare; The results show a significant relation between size of adnexal mass and probability for surgery; Ultrasound examination of adnexal masses has been proven to be accurate and safe in pregnancy; Managing ovarian cysts in pregnancy can be challenging. Goh et al. have reported similar outcomes, namely that ovarian torsion is still reported as a rare event in pregnancy and that the management of most adnexal masses in pregnancy can be conservatively managed if asymptomatic and if there are no ultrasound findings suspicious for malignancy ⁸. If a surgical intervention is needed for persistent masses with complications such as torsion Goh et al. have found that laparoscopy during 1st and 2nd trimester can be safely performed ¹. In our cohort two out of six women underwent successful major surgery during the 2nd trimester of pregnancy. One had an emergency laparoscopy for a torsion at 16 weeks of pregnancy and the other had a laparotomy at 17 weeks of pregnancy for a mucinous borderline tumour.

However, to our knowledge currently no evident guidelines exist on how to manage and follow-up ovarian masses during pregnancy. The characteristics and presentation of ovarian mass complications in pregnancy can be mimicked by similar symptoms related to pregnancy, such as ectopic pregnancy. In one of our cases a woman with a known ovarian cyst was found to have a necrotic ovary at the time of Caesarean section, despite no signs of torsion at any time during pregnancy. This only highlights how challenging and difficult the assessment of ovarian masses during pregnancy can be. Additional diagnostic examinations such as tumour markers in suspicious ovarian masses have been found difficult to interpret in pregnancy. However, there has been literature suggesting that if a mass is strongly suspicious for malignancy, it is likely that CA-125 will be severely elevated (1000-10 000) ⁷.

The strength of this study is that the data has been collected using the expertise and facilities of a tertiary referral centre in London (GSTT). The limitations of this study include retrospective data collection, small numbers of cases and loss of follow-up. Although, our study shows the benign nature of most ovarian masses in pregnancy and the ability of ultrasound to safely characterize ovarian masses, a prospective study is required to validate our results. As it is difficult to interpret ovarian cancer tumour markers in pregnancy ^7, other models, such as the IOTA Simple Rules^14,16 or the ADNEX model¹⁷ may play a role for further characterisation of ovarian masses. A prospective trial is required to validate these models in pregnancy.

INTRODUCTION

Adult Still’s disease (AOSD) is an inflammatory disorder of unknown etiology characterized by quotidian (daily) fevers, arthritis, and an evanescent rash and multi-organ involvement [1].First described in children by George Still in 1896, subsequently in 1971 Bywaters described 14 patients with similar presentation [2]. The clinical course of adult Still’s disease (AOSD) can be divided into three main patterns: monophasic (or monocyclic), intermittent, and chronic. Patients with monophasic AOSD have a disease course that typically lasts only weeks to months, completely resolving within less than a year in most patients [3]. Systemic features, including fever, rash, serositis, and hepatosplenomegaly, predominate in this group. The patient we diagnosed as AOSD, with monophasic course, went into remission after proper treatment and is symptom free even after stopping the treatment.

CASE REPORT

46 year old Indian male, non-smoker, married, nondiabetic, normotensive admitted at department of internal medicine in our hospital with history of high grade fever, polyarthritis, and skin rash for the last 4 weeks. The fever was high grade, with maximum temperature reaching 39.2°C. The patient also complained of joint pains involving the knee, ankle, wrist and proximal interphalangeal joints. There was no history of oral ulcers, morning stiffness, ocular symptoms, or contact with infected persons. In the hospital, during the febrile period, he developed macular rash mainly on chest and back [Figure 1]. On examination, the patient was sick looking, febrile-39.2°C. Chest on auscultation was normal, cardiovascular examination was unremarkable. Examination of abdomen revealed mild spleenomegaly. Neurological examination was unremarkable. Investigations revealed hemoglobin 12.7g/dl, erythrocyte sedimentation rate (ESR) 120 mm in 1st hour. Total leukocyte count-12.7 x10 9/L. Liver function showed elevated liver enzymes with Aspartate transaminase-125U/L, Alaninie aminotransferase 60 U/L, low albumin 2.3gms/dl. He was worked on lines of pyrexia of unknown origin and his blood, urine and sputum culture showed no growth. Procalcitonin level was less than 0.5ng/ml. Sputum for AFB was negative for three samples; qunatiferon gold test for tuberculosis was negative. IgM CMV, EBV, HIV, hepatitis B and C were negative malarial parasite, Widal and Brucella serology was negative. CT-chest and abdomen were normal, except for mild spleenomegaly. Echocardiogram was normal. ANA, rheumatoid factor was negative. Lactate dehydrogenase (LDH) 978 U/L, His CRP showed a progressive increase from 82mg/L to 284 mg/L, which decreased after starting steroids. His ferritin levels were 40, 000 (normal range 21.8 -274.6 ng/ml), which were reconfirmed by second sample and he had normal transferrin saturation. On the basis of his history, clinical examination and review of his laboratory investigations, diagnosis of AOSD was made. We started him on prednisolone 60 mgs daily along with Diclofenac potassium 50 mg twice daily, to which he responded and become afebrile. He was discharged with a tapering dose of steroids 5mgs weekly. He is doing well and is completely symptom free.

Figure 1: Skin Rash on the back

DISCUSSION

First described in children by George Still in 1896, “Still’s disease” has become the eponymous term for systemic juvenile idiopathic arthritis [4]. In 1971, the term “adult Still’s disease” was used to describe a series of adult patients who had features similar to the children with systemic juvenile idiopathic arthritis and did not fulfill criteria for classic rheumatoid arthritis.

The etiology of adult Still’s disease (ASD) is unknown; both genetic factors and a variety of infectious triggers have been suggested as important, but there has been no proof of an infectious etiology, and the evidence supporting a role for genetic factors has been mixed. It is uncertain whether all patients with AOSD share the same etiopathogenic factors. Proposed pathogens have included numerous viruses; suspected bacterial pathogens include Yersinia enterocolitica and Mycoplasma pneumoniae [5]. As an example of studies of the immunogenetics of ASD, in a series of 62 French patients, human leukocyte antigen (HLA)-B17, -B18, -B35, and -DR2 were associated with AOSD. However, other studies have not confirmed these findings [6].

Adult Still’s disease is very uncommon. Prevalence of AOSD is estimated to be 1.5 cases per 100, 000-1, 000, 000 people, with an equal distribution between the sexes [6]. There is a bimodal age distribution, with one peak between the ages of 15 and 25 and the second between the ages of 36 and 46. The diagnosis of AOSD is possible only by recognizing the striking constellations of clinical and laboratory abnormalities. It is also to be to remember that AOSD is a diagnosis of exclusion. AOSD has been associated with markedly elevated serum ferritin concentrations in as much as 70 percent of patients. Serum ferritin values above 3000 ng/mL in a patient with compatible symptoms should lead to suspicion of AOSD in the absence of a bacterial or viral infection. Abnormally high serum ferritin values were reported in some case reports and it was suggested that high ferritin levels may be a diagnostic marker of Still's disease [7]. Our patient showed almost all features as laid down in Yamaguchi criteria[Table 1] for the diagnosis of AOSD [8] along with a markedly high ferritin levels.

Table 1 : Diagnostic criteria for AOSD (Yamaguchi)⁸

Exclusions: Infections, malignancy, rheumatological diseases. Five criteria with at least two major criteria. ASOD: Adult onset still’s disease. WBC: White blood cell, ANA: Antinuclear antibody, RF: Rheumatoid factor, PMN: Polymorphonuclea

Non-steroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen or naproxen, help to reduce inflammation [9]. Patients with high-fever spikes, severe joint glucocorticoids, such as prednisone (0.5- 1mg/kg/day)Methotrexate has been used successfully in a small series of people to treat adult Still's disease [10]. Some patients are refractory to these conventional therapies. Tumor necrosis factoralpha (TNF) blockers include infliximab, adalimumab, etanercept, anti-interleukin-1, antiinterleukin-6 agents, and most recently anti CD20-expressing B-cell antibodies are also effective in some cases. Other experimental drugs, including cyclosporine and anakinra, have also been successful in small groups of people [9]. Interleukin 6 inhibitors like tocilzumab showed a good result in patients with AOSD resistant to other immunosuppressive agents such as methotrexate, TNF inhibitors and anakinara [11]. Even with treatment, it's difficult to predict the course of adult Still's disease. Some people might only experience a single episode, while for others adult Still's disease may develop occasional flair up or a chronic condition. About one-third of people with the disorder may fall into each of the above groups.

CONCLUSION

A diagnosis of AOSD should be kept in mind in case of pyrexia of unknown origin particularly in a patient who presents with high-grade intermittent fever, polyarthritis and skin rash of more than two weeks duration. However, the patient should be extensively evaluated to rule out other differentials of AOSD like acute or chronic infections, autoimmune disorders, vasculitis and malignant disorders. Serum ferritin values can be powerful adjuncts in making the diagnosis of AOSD [12], where they are usually higher than other inflammatory diseases. Indeed, extreme elevation of serum ferritin up to 75, 500ng/ mL has been reported in AOSD[12]. Several investigators agree that ferritin levels above l, 000 ng/mL are suggestive of AOSD while levels greater than 4, 000ng/mL are very specific for this diagnosis when accompanied by a compatible clinical picture.

The identification of dark patches on the skin may be the first indication of type 2 diabetes mellitus (DM type 2). DM type 2 is a complex heterogeneous group of metabolic conditions characterised by elevated levels of serum glucose. Causative factors are impairment in both insulin action and insulin secretion. The darkening of the skin is usually evident on the hands and feet, in folds of skin, along the neck, and in the patient’s groin and armpits.¹ The affected skin differs from that which surrounds it, and it feels velvety and also thicker. That skin may have hanging from it the small, soft, skin-coloured growths known as tags, and the area affected may be pruritic. This condition is a nonspecific dermatological disorder termed acanthosis nigricans (AN), which often occurs in patients with high insulin levels. Hud et al. (1992) found that 74% of the obese population exhibits AN.²The association of AN, skin tags, and diabetes mellitus due to insulin resistance – along with obesity in adolescents and young adults – is a well-defined syndrome.^3,4,5

High-insulin levels in the blood may increase the body’s production of skin cells, many of which have increased pigmentation that gives the skin a darkened appearance – dark patches appear on the skin. These are often the outcome of insulin receptors in the skin being triggered, causing mutations of normal tissue that are dark in colour and/or irregular in shape. The condition may be an indication that the blood sugar is persistently high. The term ‘acanthosis nigricans’ was originally proposed by Unna et al. in 1891, but the first descriptions of it were made a year earlier by two researchers working independently of each other: Pollitzer and Janovsk.⁶ Kahn and colleagues tried to clarify the link between AN and insulin resistance in 1976.⁷ Eventually, its presence became established as an indicator of insulin resistance or diabetes mellitus in obese patients.⁸ In 2000, the American Diabetes Association formally accepted AN as such.⁹ It should be borne in mind that AN must not be considered a characteristic feature of DM type 2; it is not a condition that is developed by all those who suffer from the disease.

Figures 1,2,3 - Acanthosis Nigrans

Pathogenesis

Although the pancreas produces insulin in DM type 2, the body cannot make use of it efficiently. The outcome is a build-up of glucose in the bloodstream, which may lead to high levels of blood glucose and insulin. At low concentrations, insulin regulates the metabolism of carbohydrates, lipids, and protein and may promote growth by binding to ‘classic’ insulin receptors. High concentrations of insulin may stimulate keratinocyte and fibroblast proliferation through high-affinity binding to the insulin-like growth factor 1 (IGF-1) receptors.¹⁰ In obese patients elevated IGF-1 levels may contribute to keratinocyte and fibroblast proliferation;¹¹ the binding stimulates the proliferation of keratinocytes and fibroblasts, which leads to AN.

To put this simply, AN is the outcome of a toxic effect of hyperinsulinemia. Excess insulin causes the normal skin cells to reproduce rapidly rate, and it has been demonstrated to cross the dermo-epidermal junction and reach keratinocytes. In those who have dark skin, these new cells have increased melanin. The higher level of melanin results in the creation of a patch of skin that is noticeably darker than the skin surrounding it. The presence of AN is therefore a strong indicator of increased insulin production and, therefore, it is also a predictor for future DM type 2.

When the occurrence of AN is recognised, a prediabetic person has the opportunity to become more alert to their symptoms and to take precautions in the form of diet restrictions and weight loss. This is because overweight people tend to develop resistance to insulin over time. If too much insulin is the cause of AN, it is relatively easy for the patient to counter it by changing to a healthier diet, taking exercise, and controlling their blood sugar. Obesity-associated AN may be a marker for higher insulin needs in obese women with gestational diabetes,¹²andAN has been shown to be a dependable early indicator for metabolic syndrome in paediatric patients.¹³

Autoimmunity?

Unknown autoantibodies other than insulin receptors have been implicated in AN, which may explain the effectiveness of cyclosporine treatment. Kondo and colleagues identified a very rare occurrence – without type B insulin resistance – of generalised AN with Sjögren's syndrome and systemic lupus erythematosus-like features.¹⁴Theirs was the first report of generalised AN involving an area from the mucosa of the larynx to the esophagogastric junction, accompanied by autoimmune disorder (AD) responding to systemic immunosuppressive therapy. AN skin lesions and mucosal papillomatosis were medicated with oral cyclosporine A and were accompanied by lower autoantibody titres. That was an outcome of the development of antibodies to insulin receptors in AD such as systemic lupus erythematosus.¹⁵

Raymond et al. have reported on the association of AN with disordered immunoreactivity.¹⁶ The onset of AN may precede a variety of classic ADs, and different categories of ADs may be present at the same time. If AN is an AD, DM type 2 may also represent a slow and subtle autoimmune process. AN and DM type 2 then become two different expressions of the same disease process, but the former is apparently benign and the latter is ultimately potentially fatal. Autoimmunity is a well-known pathogenic component in DM type 2. The assumption that its pathogenesis encompasses autoimmune aspects is increasingly recognised. That is based on the presence of circulating autoantibodies against β cells, self-reactive T cells, and also on the glucose-lowering efficacy in DM type 2of some immunomodulatory therapies.¹⁷ The autoimmune hypothesis of AN has the potential to modify the direction of DM type 2 research.

The symptoms of ADs are inconstant and this is in divergence to the mechanisms of antigen recognition and effector function that are alike in the response to pathogens. ¹⁸ The symptoms basically depend on the triggering autoantigen and the target tissue. In certain conditions, autoantibodies function as receptor antagonists and in other situations, they function as receptor agonist. Autoantibodies of both types can be made against insulin receptor. When they serve as antagonists as in DM Type 2, the cells of patients are unable to take up glucose, the consequence is hyperglycaemia whereas in patients with agonistic antibodies, cells deplete blood glucose resulting in hypoglycaemia.¹⁸ One wonders whether AN may be an early by-product of such an autoimmune process.

Vitiligo which is the result of depigmentation of the skin is in fact an opposite disorder to AN. Vitiligo is recognised as an AD.¹⁹Thyroid disorders, particularly Hashimoto thyroiditis and Graves’ disease, other endocrinopathies, such as Addison disease, diabetes mellitus, alopecia areata, pernicious anaemia, inflammatory bowel disease, psoriasis, and autoimmune poly-glandular syndrome are all associated with vitiligo. ²⁰ Kakourou et al identify that Hashimoto's thyroiditis is 2.5 times more frequent among children and adolescents with vitiligo than in a healthy age- and sex-matched population and it usually follows the onset of vitiligo. ²¹

As in the case of other ADs, vitiligo susceptibility may involve both target organ-specific genes and immune response genes. ²² The autoimmune theory suggests alteration in humoral and cellular immunity in the destruction of melanocytes of vitiligo. ²³. Vitiligo lesions have an infiltrate of inflammatory cells, particularly cytotoxic and helper T-cells and macrophages;histological evidences further back up an autoimmune aetiology. ²⁴ Like AN, Vitiligo is thus gene linked; immunity derangements may be providing the matrix and genes are the craftsmen in both conditions.

Vitiligo occurs more commonly in DM Type 1. A few recent studies have revealed its increased incidence in DM Type 2. ²⁵ These may be isolated case studies, but offer new insight into the pathogenesis of DM Type 2. There is a logical thread running between the autoimmune assertion of AN and its depigmentation counterpart (vitiligo) which is recognised as an AD. If AN is proven as an AD, the AD hypothesis of DM Type 2 becomes more binding. The autoimmune process of AN warrants further consideration and further study is needed to confirm or falsify the hypothesis of an autoimmune spectrum disorder between AN, vitiligo and DM Type 2.

Even though the common assumption that bacteria flora occupying human body outnumber the body cells has been proven wrong, their revised ratio of 1:1 is still astounding.²⁶ The exact role of the resident microbial colony in human body is unclear. There are less ADs observed among the hunters’ tribal population of Tanzania whose faecal matter contain more varieties of microbes than people in developed countries. ^27,28 This is an observation that need further verification. It is possible that the occupied microbial army may be maintaining the harmony among the human body cells from attacking each other and serving as moderators. Now that anti-autoimmune activity in molecules produced by parasites have been confirmed in haematology lab, these findings may have clinical significance. The aetiology of ADs is multifactorial. Genetic, environmental, hormonal, psychological stress and immunological factors are all considered important in their development. I content that the clue to the mechanism of development of certain ADs and ways of counteracting them may be embedded in the bacterial colony and their interaction with human cells.

International studies

A pilot study by Bhagyanathan and colleagues demonstrated that children with AN have a high incidence of insulin resistance.²⁹ They posit that the detection of insulin resistance in children may present an opportunity to prevent the onset of microvascular changes before the development of DM type 2. Once DM type 2 by hyperglycemia is diagnosed it may be too late for that. Insulin resistance is one of the mechanisms involved in the pathogenesis of DM type 2; therefore, early recognition of insulin resistance is paramount in the prevention or delay of the onset of diabetes. Their study was of 62% of children who had AN alongside high insulin resistance. In children with AN and a high BMI, the incidence of insulin resistance was about 80%. This is evidence that the easily detectable symptoms are of value in the screening of children who are at high risk of developing DM type 2. Bhagyanathan et al. conclude that AN has potential as a screening method because those who have high insulin resistance as well as AN are at high of future DM type 2.

An earlier US study, by Brickman and colleagues, had yielded somewhat similar results.³⁰ This involved 618 youths from different ethnic groups at nine paediatric practices. They were aged from 7 to 17 years. A survey was made of their demographics and their family history with regard to DM type 2, and their weight and height were also measured. AN was scored and digital photographs of their necks were taken. AN was identified in 19%, 23%, and 4% of the African American, Hispanic, and Caucasian youth respectively. It was also found in 62% of those studied who had a BMI greater than the 98th percentile. Using multiple logistic regression, the researchers found that the level of BMI, the presence of maternal gestational diabetes, female gender, and not being Caucasian, were all independently associated with AN. AN was common among the overweight young people and was associated with risk factors for glucose homeostasis abnormality. Brickman et al. concluded that identification of AN offers an opportunity to advise families about the causes and consequences of the condition. ³⁰ That has the potential to motivate those with responsibility for the young people to encourage and effect healthy lifestyle changes that decrease the risk of the development of DM type 2 and cardiovascular disease.

In their research in India, Vijayan et al. determined that BMI, waist circumference and AN are three physical markers for the recognition of insulin resistance in children.³¹ They conducted a cross-sectional school-based study in a semi-rural environment in the state of Kerala, which has become known as the diabetic capital of the country. Their study encompassed 283 children between the ages of 10 to 17. The prevalence of insulin resistance was 35%; this estimate was arrived at by using a homeostasis model assessment of insulin resistance (HOMA-IR). Of the children studied, 30% had a waist circumference above the 75th percentile and 18.7% had a BMI above 85th percentile. AN was diagnosed in 39.6% of the population studied. A significantly high prevalence of insulin resistance was observed among the children with a waist circumference exceeding the 75th percentile, a BMI above the 85th percentile, or a diagnosis of AN. The most sensitive physical marker of insulin resistance was AN (90%) and the most specific was BMI (91%). By combining these parameters their sensitivity may be increased to 94% and their negative predictive value to 96%. Vijayan et al. conclude that these easily recognisable physical markers are an efficient warning of insulin resistance among children.

Acanthosis nigricans in different conditions

AN is not a disease, but a symptom of disease. A high prevalence has been observed recently, and there are a number of varieties. These include benign, obesity associated, syndromic, malignant, acral, unilateral, medication-induced, and mixed AN.^32,33 It has been established that AN may occur in a number of conditions, and a brief discussion of these is appropriate for this paper. Different types of AN are listed in Table 1. It often appears gradually in the prediabetic state, but abruptly in malignancy.

AN may be triggered by a plethora of medications, such as birth control pills, human growth hormones, thyroid medications, and even some bodybuilding supplements. All these medications may cause changes in insulin levels. Medications used to ease the side effects of chemotherapy have also been linked to AN. In most cases, the condition clears up when the medications are discontinued. In rare cases, AN may be caused by gastric cancer (especially gastric adenocarcinoma) or an adrenal gland disorder such as Addison’s disease. Hypothyroidism, Cushing’s disease, and polycystic ovarian disease are also common causes of AN. ^34,35

When AN is present without any identifiable cause in middle-aged and older patients with extensive skin findings, internal malignancy needs to be ruled out. AN has been reported in association with many kinds of cancer, by far the most common being an adenocarcinoma of gastrointestinal origin. In these patients it is a rapid-growing dermatological pigmentation disorder. The skin changes are typically more extensive and severe than those seen in benign AN. Findings may include thickening, unusual roughness and dryness, and/or potentially severe itching (pruritus) and irritation of the skin regions affected. Pigmentary changes may be more pronounced than those observed in benign AN and they are not restricted to areas of hyperkeratosis. Malignant AN is frequently associated with the mucous membranes and with distinctive abnormalities of the oral (mouth) region. For example, reports indicate that the lips and the back and sides of the tongue may have an unusually ‘shaggy’ appearance, sometimes with elevated, wart-like, non-pigmented tissue growths (papillomatous elevations). Malignant AN is also commonly characterised by wart-like thickening around the eyes, unusual ridging or brittleness of the nails, thickening of the skin on the palms of the hands, hair loss, and sometimes other symptoms. Investigators have reported that the development of malignant AN may occur as much as five years before the onset of other symptoms, although the time span before malignancy is typically of shorter duration.

Table 1. Different types of acanthosis nigricans

Genetic links

It is worth noting that certain types of AN may be genetically linked.³⁶ The interaction of genes and the environment is not clearly understood and the different variables of DM type 2 are not established. It is a heterogenous disorder and there is a general consensus that diabetic comorbidities may be the outcome of genetic and environmental susceptibilities.^{37,38,39,40,41} Such factors may have an influence independently or in combination with one another that brings about hyperglycaemic conditions.It would be interesting to explore the possibility of a link between the diabetic genes and the AN gene. DM type 2 may be potentiated by poor quality of insulin or decreased production of insulin, and the distinction between those manifestations is not well recognised. The controversy concerning the relative roles of insulin deficiency and insulin resistance in DM type 2 continues to be unresolved.⁴² Despite the early demonstration that obese people have elevated plasma insulin concentrations, many studies over the years have failed to control satisfactorily for the influence of obesity.⁴³ Another difficulty with the interpretation of plasma insulin concentrations is that sustained hyperglycaemia may have detrimental effects on insulin secretion.⁴⁴ Diabetes mellitus affects every cell of the body, and therefore it affects the beta cells of the pancreas in turn. The spiralling effect of hyperglycaemia adds to the malfunctioning of beta cells, and that results in impaired quantity and quality of insulin. Only a subset of diabetic patients shows AN, and other groups of obese diabetic patients do not develop AN. AN is linked with higher insulin production and obesity, whereas AN may not be present in diabetes with a reduced quantity of insulin. The presence of AN may serve as one of the biological markers to determine subtypes of DM type 2.

The incidence of AN varies in different races, which is evidence that AN may have a genetic contribution – indeed, it has been regarded by some as being strongly influenced by genetic factors. It is thought to be autosomal in nature. AN is common among African-Americans, Hispanics, and American Indians, but it is rare among white people.^45,46 A study from the USA reports the prevalence of AN as 3% among Caucasians, 19% in Hispanics, and 28% in American Indians. ⁴⁷ More recently, studies from Sri Lanka and south India show the prevalence of AN as high as 17.4% and 16.1% respectively in the adult population in general.^48,49

Type 2 diabetes mellitus and schizophrenia

DM type 2 is relatively common among people who have mental health issues. Increased risk for cardiovascular disease and other serious illnesses related to insulin resistance – for example, certain epithelial cell carcinomas, AN, and polycystic ovary syndrome – are long-term concerns associated with the cluster of metabolic abnormalities stemming from insulin resistance. These are often referred to as the metabolic syndrome.⁵⁰ Impaired action of insulin in patients with schizophrenia was reported over fifty-five years ago and later confirmed in Australia.⁵¹ The prevalence of DM type 2 in patients with schizophrenia was found to be higher than it was in the general population, even before antipsychotic medication was in widespread use.

The mechanisms underlying the relationship between schizophrenia and diabetes remain unexplained. The present author has argued in favour of the autoimmune hypothesis of a subset of schizophrenia.^52,53 The proposal is that if AN is an AD, it may be co-existing with DM type 2, or the DM type 2 itself may even be an extension of the same autoimmune process. In other words, there may be a continuum of pathological process between AN and DM type 2. It follows that schizophrenia sufferers may have a predisposition to develop DM type 2; schizophrenia may even be considered as a clinical surrogate of DM type 2.

When AN occurs in a schizophrenic patient, they sometimes develop a delusional misinterpretation of the condition, such as that it is a result of skin cancer or even a manifestation of an external agency. Such situations may result in severe anxiety. Schizophrenia is frequently associated with poor lifestyle choices on the part of the patient, such as a diet high in fat, reduced levels of physical activity, and high rates of smoking-all of these may contribute to the development of a metabolic syndrome and insulin resistance.^54,55 It is worth considering investigation into the early warning signs for DM type 2 – including the AN – before commencing a patient on antipsychotic drugs that lead to a metabolic syndrome.

It is now well recognised that patients treated with clozapine or olanzapine are more often classified as having DM type 2 or impaired glucose tolerance in comparison with patients treated with other second-generation antipsychotics. Clozapine increases the risk of diabetes if there is a history of pre-existing diabetes or a family history of diabetes. According to a US study, the risk is higher if the patient is African-American or of Hispanic origin. Such patients may need close blood sugar monitoring during the initiation of clozapine treatment. I contend that if a patient already has AN, weight-increasing antipsychotics should be avoided. Even though aripiprazole is the most metabolic-sparing agent among the second-generation antipsychotics, Manu et al. report a case of AN in a patient treated with it. That patient did have a family history of DM type 2, which adds to the interest of the case.⁵⁶

Diagnosis and treatment

There is no specific treatment for AN. Treatment is directed towards the specific symptoms that are apparent in each individual. It should be borne in mind that such treatment may require the coordinated efforts of a team of medical professionals. Correcting the underlying disease improves the skin symptoms. Steps that may be taken, depending on what the disease is, include correcting hyperinsulinemia through diet and medication, encouraging the loss of weight in those with obesity-associated AN, removing or treating a tumour, and discontinuing a medication that causes AN. The control of obesity contributes significantly to reversing the whole process, essentially by reducing both insulin resistance and compensatory hyperinsulinemia. However, the pigmentary changes may persist. In drug-induced AN, offending medicines should be stopped. In hereditary AN, lesions tend to enlarge gradually before stabilising and/or regressing on their own.

For those with AN, the recommended treatment may include the use of certain synthetic, vitamin A-like compounds (retinoids). For individuals with malignant AN, disease management requires treatment by oncologists. Reports indicate that AN has improved with therapy used to treat underlying malignancies and has reappeared with tumour recurrences. Other treatment for this disorder is symptomatic and supportive. The treatments considered are used primarily to improve appearance, and include topical retinoids, dermabrasion, and laser therapy. The final outcome of AN varies depending on the cause of the condition. Benign conditions, either on their own or through lifestyle changes and/or treatment, have good outcomes. The prognosis for patients with malignant AN is often poor as the associated cancer is often advanced.

AN may be diagnosed on the basis of thorough clinical evaluation, identification of characteristic physical findings, a complete patient history including medication history, a thorough family history, and various specialised tests.⁵⁷ The age at detection will vary, depending upon the form of AN present and on other factors. For example, benign forms of AN often become evident during childhood or puberty. It is less common for benign AN to be apparent at birth or to develop in adulthood. The latter cases most typically involve AN in association with obesity.

In individuals with skin changes that suggest AN, diagnostic assessment may include various laboratory tests. Examples are the glucose tolerance test and the glycated haemoglobin (HbA1c) test. Additional laboratory studies or other specialised tests may also be utilised in diagnosis in order to help detect or rule out certain other underlying disorders – including a number of endocrine and autoimmune conditions – that may be associated with AN. In addition, in some instances, particularly where the patient presents with signs suggestive of malignant AN, testing may include biopsy and microscopic evaluation of small samples of skin tissue affected.

The onset of malignant AN usually occurs after the patient reaches 40 years of age. Various factors may be indicative of malignant AN in association with an underlying cancer. These include symptom onset in adulthood that is not associated with the use of particular medications, obesity, a positive family history, and certain underlying disorders known to be associated with AN. It is rare for malignant AN to develop during childhood. In such instances, warning signs may include skin changes that progress rapidly and also involvement of the mucous membranes.⁵⁸

AN may be metaphorically linked to the dark pigmentation that appears on the skin of the ripe Sharon fruit. Sharon fruit is the trade name for a variety of persimmon that is grown in Israel. In the fruit the dark patches on the ripe and sugary fruits are the result of condensed tannins. Insulin-resistant AN may be referred to as the Sharon fruit sign in order to emphasise the diagnostic value of the condition. It has been suggested that the official terminology for AN is inappropriate for a significant warning of an increasingly common disease for which early diagnosis is imperative. Because the complex name may have a negative impact on its identification by both clinicians and patients, a less formal term is in use among some of those who are concerned with patient care. It must be borne in mind that AN, otherwise Sharon fruit sign, manifests only in those with the insulin-resistant condition and should not be considered a characteristic feature of DM Type 2. Identification of the Sharon fruit sign may be helpful in the early diagnosis of DM type 2.

Discussion

Diabetes puts an enormous burden on patients, their families, and the health-care system. Detection of the disease at an early state using physical markers and instituting preventive measures will reduce the economic strain on society to a great extent. According to the latest global data from the World Health Organization (2016), an estimated 422 million adults are living with DM type 2 and diabetes prevalence is increasing rapidly.⁵⁹ In 2013 the International Diabetes Federation estimated that 381 million people were living with diabetes.⁶⁰ That number is anticipated to almost double by 2030.⁶¹ About 3.8 million people in the UK have DM type 2, and the charity Diabetes UK has made predictions that it may become as high as 6.2 million by 2035/36.

Most often a diagnosis of DM type 2 is made only when such symptoms as loss of weight, polydipsia, and polyurea have become manifest. By that time the damage to the body may have already come about. Complications arising from diabetes cover the entire area of medical science, so early detection is crucial. Intervention at the prediabetic stage helps to arrest the progress of this condition. AN may herald DM type 2, endocrinopathies, and malignancies. This cutaneous disorder is easily detectable and highly useful in the early detection of the disorders associated with it. Early screening for AN in preadolescent and adolescent people would provide a relatively simple, inexpensive, and non-invasive tool for identifying those young people who have hyperinsulinemia and could benefit from early intervention. That would prevent the development of DM type 2. Young people tend to be reluctant to undergo traditional screening measures and definitive diagnostic tests as they find them invasive and unpleasant.

A sedentary life style and unhealthy dietary habits – as well as the side effects of antipsychotics – make chronically ill psychotic patients more vulnerable to DM type 2 than the general population. Long-standing detained patients in particular are restricted in their mobility and may become more prone to obesity and insulin resistance. It is not clear whether the pathogenesis of psychosis itself has a diabetogenic effect. It is evident that because of the high incidence of DM type 2 among mental health service users, psychiatrists need to become more alert in the diagnosis, management, and prevention of the complications of DM type 2.

Introduction

Medical scientists who espouse a strict biological model of the mind tend to care less about the prolongation of life than do those who have faith in higher authority.¹ The prevailing reductionist model of mind has recently been challenged effectively.^2,3,4,5. That has led to a position in which there is some justification for claiming that there is scientific evidence to enable a suspension of disbelief in life after death. ⁶ Medical profession should respect the theology veiled in thanatology and should be careful not to become instrumental in creating a culture of death; alleviating suffering is not by eliminating the patient.

In the absence of spiritual conviction, human suffering lacks deep meaning and death is regarded as the ultimate tranquilliser. Prolonging life at any cost may be perceived as a worthless endeavour. To counter that, without suffering evolution would not take place and human consciousness would fail to expand. Without stress and struggle the spirit buds to which we may be likened would not mature and grow leaves and fruit, and our characters would not develop; we would lead the lives of lotus-eating sybarites. ⁷

Evidence for discarnate survival

According to those who are sceptical about after-death survival, there is only as much evidence to justify belief in life after death as there is for the historical existence of dinosaurs. Some scientific researchers however argue that there are compelling reasons to support those who are proponents of belief in life after death. Dr Vernon Neppe, a neuropsychiatrist turned parapsychologist, has declared that the combined body of evidence for discarnate survival is overwhelming – so great that it may be regarded as scientifically cogent.⁸ This emerging scientific view, coupled with the wisdom of the faith traditions, challenges the rationality of supporting assisted suicide. The following are examples of evidence for discarnate existence that are commonly cited:

• clinical death experiences
• pre-death visions
• shared death experiences
• collective apparitions
• some forms of mediumistic incidents, particularly ones that involve cross-correspondence, drop-in communications and physical phenomena
• children’s memories of previous lives
• electronic voice phenomena
• instrumental trans-communications
• transplant cases
• Scientifically studied Marian apparitions

The list is becoming longer as survival research progresses. Encouraged by the success of afterlife experiments with mediums,⁹ the multi-specialist professor Gary Schwartz of Arizona University claims to have invented a device to communicate with discarnate spirits; the holy grail of survival research that could possibly offer a fool proof scientific evidence of afterlife existence,¹⁰ but also takes account of all the potential negative consequences. He claims to have worked with black boxes in his laboratory, using a software programme that has generated proof that there is a spirit world by measuring light. ¹¹It appears that he has developed a technique whereby faint light can be detected in a totally dark box. Measurements are taken at the beginning of an experimental session, and then a specific “hypothesized spirit collaborator” is asked to show a “spirit light” in the box and a second reading is taken. The finding is that instruction for specific spirits to enter a light sensing system was associated with reliable increase in the apparent measurement of photons. Such a curious result means that these communicating spirits are able to hear, respond and produce light in an otherwise dark enclosure. ^12,13 The conclusion is that survival research opens up new vistas which seem much more important than cosmology or quantum electrodynamics.

Scientifically examined Marian apparitions are a recent addition to the evidences for discarnate existence. ¹⁴ Mainstream scientists seem never to have attempted to develop the conceptual tools and vocabulary needed to investigate the possibility of post-mortem existence. It may be that science will not accept the possibility of discarnate survival without a new theory of physical reality. In the early part of the twentieth century the prevailing view of scientists was that there was no possibility whatsoever of proving the existence of life after death. Over the years that have passed since then attitudes have evolved, and in the world, we are now in it is asserted by some researchers that there is scientific evidence for the existence of life after death. Some of the evidence relating to discarnate existence may not however satisfy the criteria of the physical sciences since the latter are based on speculative science and court room logic.

Paradigmatic shift

Demonstrating post-mortem existence as an irrefutable phenomenon is a route to establishing empirically that humans have a higher consciousness. Unfortunately, in survival research there are many phenomena that have multiple possible explanations, and these augments add to the complexity of this immensely significant area of scientific enquiry. All the types of evidence postulated as supporting discarnate survival are simultaneously a form of evidence of a non-biological component that operates in association with the brain. The existence of a non-biological component indirectly proves the possibility of survival after physical extinction. A huge paradigmatic shift towards non-reductionism is now taking place in the cognitive sciences – consciousness is no longer considered an epiphenomenon of brain activity, but asthe designer and prime mover of the material body. Nowadays, some mainstream scientists are themselves paradoxically trying to debunk mainstream science.

Suicide victims

Through suicide, a person is simply changing the location of their suffering. While wrapped in the physical planet by space and time, we are in an advantageous position for inducing personality changes swiftly, whereas in the timeless state of discarnate existence changes are sluggish and personality development is much slower. Contemporary data for survival research may be congruent with the wisdom of the faith tradition. ¹⁵ To use a simple analogy for this, carrying out assisted suicide is like destroying the shell of a pupa and forcefully freeing it in a premature state. Such a pupa will not be able to fly about like a butterfly. It is arguable that a person subjected to violent death – as in the case of suicide – may not be able to enjoy the beauty of God’s grand other-worldly dimensions until they have become spiritually compatible with them. They have to navigate through the physical plane like wingless birds. ¹⁰ To look at this way, if fruit that is unripe drops from a tree, it will be sour. Suicide breaks a solemn law because it deprives the conscious self of the natural growth that life in a physical body can best provide.⁷The Chinese saying “One day of earthly existence is not equivalent to a thousand days of ghostly existence” is a statement of the sanctity of terrestrial life.

Lord Alton has campaigned against the Assisted Suicide Bill of 2014 since its inception. Referring to his dying father’s account of how he had seen his own brother, a member of the Royal Air Force who had died in the Second World War, Lord Alton argued that a forced death, as opposed to a natural one, may deprive a person of their “healing moment.” ¹⁶ A graceful and natural death may be supposed to be accompanied by benign caretaker spirits with exuberant love who assist those who are dying by making them comfortable for the big transition.^17,18 A person who terminates their own life prematurely may not be so fortunate as to get such benevolent assistance from the spiritual realm. Most hospice workers are very familiar with departing and death-bed visions such as that described by Lord Alton. Furthermore, it has been suggested that beings from the imperceptible spiritual sphere who assist in delivery from the terrestrial plane have a role in such matters as the timing of death, and it is arguable that their part in what happens should not be impeded by intervention.

It appears that human brain is designed to have some doubts about discarnate survival for some reason and a fool proof evidence of post-mortem existence may have its down side in that somebody who is fed up of life might use it to justify ending his earthly life voluntarily. ¹⁹An ultra-optimistic view of discarnate life is spiritually counterproductive and such an over optimism could be seen as a justification by the patient and carers in the decision making of assisted suicide. In a weak moment of extreme psychological or physical sufferings, such a belief can also become the final rationalization for ending one’s own life voluntarily. In my own clinical practice, I have come across suicidal patients telling me, “It will be always better on the other suicide.” A belief in discarnate existence based on parapsychological proof alone did not deter one such patient making a serious suicidal attempt

End-of-life concerns

The evening of life was considered as a great opportunity for spiritual, emotional and psychological growth and a celebration of one’s life journey. These are also times to harvest the wisdom of yesteryears and share them with the succeeding generation. Spiritually enlightened people consider this to be the time to conquer the fear of death. Fear of death is not the fear of the physical pain of death, but the fear of truthful self-judgement after death. Recent observations in thanatology favour a belief in post-mortem self-assessment and appraisal. For some, it would be voluntary or assisted, whereas for others it could turn out to be forced upon them. The final phase of life is the time to settle the errors committed against fellow beings that have not been remedied in life. Fortunately, modern medicine has prolonged this period, which grants an opportunity for most people to experience conscious ageing. Sadly, traditional attitudes towards the evening of life have changed in today’s youth-obsessed culture. For some, medical procedures have extended life and made dying a lingering process rather than a sudden event, and have contributed their own problems. For several reasons, terminally ill people who are in crisis may wish to die rather than being kept alive longer (Table 1).

From an evolutionary point of view, there can be only a survival instinct – no Freudian death instinct. Avoiding death rather than seeking it is a natural human urge and the fear of death may affect every individual action. The very concept of euthanasia is totally against the human make-up and is entirely artificial. Assisted dying and assisted suicide are the same thing when a member of the medical professional gives a lethal drug to a person so that they can take their own life. Euthanasia is different; it happens when, for example, someone injects a lethal substance into a patient. Involuntary euthanasia refers to a situation in which the patient has the capacity to give consent, but has not done so; and in non-involuntary euthanasia a person is unable to give consent, for example because of dementia or being in a coma. Mercy killing is claimed to be a compassionate act to end the life of a patient.

Table 1: End of Life Concerns

1. Losing autonomy
2. Less able to engage in activities making life enjoyable
3. Loss of dignity
4. Burden on family, friends/caregivers
5. Losing control of bodily functions
6. Inadequate pain control or concern about it
7. Financial implications of treatment

Moral and ethical issues

It has been observed that the risk of suicide is higher among people with a family history of suicide. Family culture and genetics may account for the increased incidence of suicide in such situations. Assisted suicide would create a trail in the culture of more families and more succeeding generations would perhaps be at increased risk of considering suicide as a serious option at a time of crisis. Kevil Yull (2013) comments that changes in the law of assisted suicide would have an additional impact on those left behind, because of their effect on the moral connections, assumptions and accepted responses to situations on which we base our relationships with fellow human beings and establish ourselves in the world. ²⁰ He argues that the legalisation of assisted suicide would undermine freedom instead of promoting freedom of choice, and also that the proposed safeguards and regulations would breach the privacy of the death-bed.

Assisting someone to kill themselves is assisting them in murder. According to all the major faith traditions, life is a gift from God and ending it is like throwing a precious object back to the giver. All spiritual traditions teach and believe that bringing the human heart to a standstill is God's business (Table 2). There are patients who assert that even if all their limbs were amputated, they would still want to hold on to the treasured gift of life. It is very difficult to define what unbearable suffering is; extreme suffering is a subjective matter that it is not possible to separate from an individual’s outlook on life. A fundamental question is that of who would be the one to pronounce a verdict on when suffering is intolerable – the patient or medical personnel?

Laws are not precision-guided arrows and they may become perverted. In a world full of violence and crime, assisted suicide is unsafe and could be exploited. There would be many unintended consequences. For reasons of public safety alone, some people oppose assisted dying. Financial abuse by relatives of the elderly seems to be becoming more common; those with a vested interest could be tempted to put an inheritance before life.

The regulation of assisted dying has been modified in recent times in some countries, an example being the Netherlands in 2014. There it is now lawful to kill a patient without their consent, and euthanasia and assisted suicide may be offered to people with mental health problems (consensus with the family is required in all these situations). Inboth Belgium and the Netherlands the euthanasia of children is legal with family consent (in Belgium there is no age limit; in the Netherlands the child must be 12 or above and must give consent). In Belgium blind adults who were developing further problems were granted euthanasia at their own instigation a few years ago. There is public concern about collaboration between euthanasia teams and transplant surgeons in Belgium.

Table 2: Medical dilemmas

1. Assisted suicide promotes a human right to commit suicide and gives wrong messages to suicidal patients in psychiatry. 
2. It undermines the Universal Declaration of Human rights and strikes at the foundations of all spiritual values. 
3. It is hard to define unbearable sufferings. 
4. Assisted suicide has many unintended consequences. 
5. Death with dignity could deteriorate as death with indignity. 
6. It might permit the unlawful killing of innocent people in certain circumstances. 
7. It is founded on unethical principles-survival of the fittest.      

In 2013, 1.7% of all deaths in Belgium were hastened without the explicit request of the patients. ²¹ Professor Cohen Almagor, the author of 2015 report on euthanasia in Belgium stated that the decision as to which is no longer worth-living is not in the hands of the patient but in the hands of the medical personal. ²² More than 500 people in the Netherlands are subjected to euthanasia without their consent.²³ Data from Oregon where assisted suicide was legalised in 1977 shows that the top five reasons people choose assisted suicide are not because they are suffering from a terminal illness and 49% stated that feeling like a burden and a fear of loss of control are among the main reasons for choosing assisted suicide Oregon .²⁴ In Washington state in 2013, 61% of people who were killed in assisted dying said that being a burden was a key factor for their choice of death. ²⁵

Medical Dilemmas

Majority of British medical practitioners are against assisted suicide. ²⁶ A 2013 survey showed that 77% hold the view that they would oppose a change in the current law to allow assisted dying, 18% favoured the RCGP moving to a neutral position, and only 5% favoured a change in the current law. They opined that a change in the law would make patients afraid of their doctor and would alter doctor –patient relationship, and would make vulnerable patients most at risk from assisted dying. According to Marris bill, some people should be given help to die meaning that some lives are worth less than others. Vulnerable people would feel pressurised to choose death and could be killed without their explicit consent. GPs feel that it is their privilege to protect the disadvantaged and vulnerable people of the society.

Assisted dying would lead to less focus on investment in palliative care. The RCGPs also cautioned in the survey that a change in the position of the law makers would become like abortion legislation, which started as something for extreme circumstances and is now effectively on demand. They are also anxious for the fact that legalisation of assisted dying would make it impossible to tell the real reason why patients decided to die, because illness can cause people to become depressed and frightened. As debate on assisted suicide has become hotter, in clinical practice suicidal patients have already started enquiring about the prospects of assisted death.

Thanatology

Medical sciences have not advanced enough in matters of death to offer details to make informed choice for those who want to die voluntarily and thanatology is only a fledgling science. Thanatology is the scientific study of death and investigates the mechanisms and forensic aspects of death, such as bodily changes that accompany death and the post-mortem period, as well as wider psychological, parapsychological and social aspects related to death. They are not particularly interested in the meaning of life and related philosophical issues, but this is an area where science and philosophy not be separate. In recent years, studies of parting visions by Elizabeth Kubler Ross and Raymond Moody’s NDE studies. ^{27,28,29,30,31,32} have given a spiritual dimension to thanatology. Theology and Thanatology are two major corpuses of human wisdom that cannot but overlap. Assisted dying would probably become also an issue of forensic sciences.

It is the job of the doctor to keep the patient alive whereas it is the job of the psychotherapist to have a sense of a bigger picture. ³³ People wanting to hasten death should also have the choice of receiving pastoral and psychotherapeutic assistances to distract themselves from their preoccupations of death and allow nature take its own natural course. New generation psychotherapists will have to be well versed in all aspects of death related sciences. Thanatology has a rightful place in medical studies, but I content that medical professionals need not to be unduly concerned about the different forms of afterlife existences, the borderland between religion and thanatology. Medical professionals are expected to be above religion and politics. Thanatologists now fear that if assisted suicide is legalised, they might be pressurised to slip from the original goal of acquiring more knowledge of human dying to serve the dying into the pursuit of death.

Concluding Remarks

Assisted suicide or euthanasia is incongruous with the theological view that it is the weakest and the vulnerable who can teach us the values of life and the concepts of euthanasia or assisted suicide have an indirect message of discarding them. The right to die would soon deteriorate as duty to die to prepare room for fittest ones. Instead of looking for reasons to live, people will be looking for reasons to die. What is need is better understanding of death process and advancements in the palliative care of the terminally ill, rather than doing away with them. Until we know more about the death process, assisted dying debate should be kept on hold. More research in palliative care and allowing people to die naturally with dignity should be the concern of medical profession.

Evolution may be taking place in biological and spiritual streams and they are interconnected: biological sufferings maybe aiding spiritual evolution. ³⁴. From a philosophical perspective, the rationale of terminal sufferings is to help the individual to disengage from the “pleasant illusions” of earthly life. The debate of assisted suicide raises the question whether human beings are mere electrical animals, quantum beings or fundamentally spiritual personalities-humans maybe all the three.The sanctity of human sufferings need to be brought into the equation of assisted suicide discussion. Assisted suicide would only add to the growing violence in the present world that could do with reintroduction of principles of non-violence.

USA may have better legal infrastructure to negate the unwanted and unintended errors of assisted suicide, but in many third world countries, where there is no such legal infrastructure, the procedure would easily get dishonoured. Oriental religions as well as Abrahamic faith traditions are opposed to ending life voluntarily. In general, all faith traditions believe that life that is nearing the biological end need not be preserved at all costs and that one does not have to go to extraordinary lengths to preserve a terminally ill person’s life. This means, for instance, that while a terminally ill person should not be denied basic care, he or she could refuse treatment that might prove to be futile or unduly burdensome for the dying person - passive voluntary euthanasia.

A scientific belief in after death existence is not without its pitfalls unless it is accompanied by the spiritual corollary of sanctity of earthly life. Science alone cannot highlight the sanctity of life; Divine standards are helpful in comprehending the sacredness of life. In fact, science has taken us to a cross road with Professor Schwartz’s new instrumental communication and it is time mark the boundaries of healthy survival research and the unhealthy ones.

Introduction

The Royal College of Psychiatrists (RCPsych) launched its five-year Recruitment Strategy in 2011 aiming to achieve a 50% improvement in recruitment to core psychiatry training and a 95% fill rate of posts by the end of the five-year campaign ⁽¹⁾. The primary focus of this campaign was on recruiting UK medical graduates.

Two of the Strategy’s main aims were to highlight good practice in undergraduate teaching and to improve the teaching skills of psychiatrists to inspire and influence medical students during their psychiatry curriculum.

The Strategy stressed the importance of good clinical placements in psychiatry and recommended that medical students should ideally be placed only with ‘the best teachers and welcoming teams’ avoiding colleagues who are disillusioned with psychiatry or not happy with their jobs.

It is therefore essential that psychiatrists and other clinicians play an important role to improve the medical students placement in their workplace in order to give the students a positive expereince of this speciality and hopefully promote it as a future career option.

Background

Fourth-year medical students from the University of East Anglia (UEA) spend two months rotating through various mental health services as part of their clinical placement in the Mind Module (also known as Clinical Psychiatry or Module 11).

As part of this rotation, students are placed in Old Age Psychiatry for six days over a two-week period. They shadow clinicians in two community teams, two inpatient wards and the Electro-Convulsive Therapy (ECT) clinic. All of these teams are based at the Julian Hospital in Norwich.

The students are encouraged to talk to patients and carers and perform basic clinical tasks such as mental state examination and risk assessment. Table 1 summarises the learning outcomes for students during their placement.

After each rotation, each student is asked to complete a feedback form regarding their placement. This feedback helps the module leads and clinicians to improve the students learning experience.

Before the implementation of our placement improvement project, the students did not feel that they were meeting their learning outcomes. Table 2 summarises the major areas that needed improvement.

Student dissatisfaction with clinical placements is not unique to psychiatry. Research has shown that educators and learners face significant challenges when teaching and learning take place in any clinical setting. See Table 3 for a summary.

One of the biggest challenges of teaching in clinical settings is how to provide a welcoming and supportive learning environment in a busy and time constrained practice. We found that one of the main reasons for clinicians to be reluctant to have students shadowing them is the challenge of providing a dual role of caring and teaching simultaneously.

The placement improvement project

The improved structure of the student's’ placement in Old Age psychiatry was based on the tenet that clinical placements should provide various clinical experiences that include interaction with patients and professionals from various grades in addition to face to face teaching in small groups ⁽³⁾. The authors took over full responsibility for coordinating the students’ placements and liaising with the various supervising clinical teams. This ensured clear leadership and consistency in organising the placement.

The improved placement structure started in October 2015 with the first cohort of medical students coming to their clinical placement after the summer break. Table 4 gives a summary of the changes implemented.

The information pack sent to the students before the placement contained information about the hospital environment (location, map, parking, travel arrangements, key codes and useful contact numbers) and a detailed timetable (and email address) of the clinician supervising the student each day during the placement. Also, it included useful information about the mental state examination and the Mental Health Act, information that had been requested previously by medical students.

Sending information before the placement has been shown to be beneficial in students’ electives ⁽⁴⁾ and this is especially important in psychiatry which can be experienced as less structured than other medical specialities and where students are required to travel to various hospitals and clinics bases. As a result, students felt that they were expected and had a clearer sense of where they should be and who would be supervising or teaching them. Later student feedback reported that these changes had contributed directly to an improved learning experience.

The timetable design ensured that every student would have the chance to experience working in several settings in Old Age Psychiatry, including community, inpatients, ECT and the Memory Clinic. It was also noted that a two-week placement in any psychiatric team could not easily give a student a sense of patient ‘recovery’. It was, therefore, decided that students see a patient who had been discharged from the ward, e.g. with the care coordinator.

The rota of the ‘Meet and Greet’ event on the first day of the placement ensured that the workload is spread among the clinicians and helped sustain the necessary levels of enthusiasm and energy. Previously, this task had repeatedly fallen to just a few clinicians.

The participation of all professionals in the clinical team in supervision and teaching helped the students to better understand the different roles of clinicians within the multidisciplinary team and enriched their learning experience. To achieve this, we attempted to allocate sessions with a clinical psychologist, care coordinators, memory assessment nurses and members of the intensive support team. It also had the bonus of ensuring that the workload of teaching was spread more equally among clinicians.

Attendance at ward rounds and community MDT meetings could be a valuable experience but only if the process is explained, and – in the ward round – the student is briefed on the clinical history and background of the patients. For these reasons, supervising clinicians were reminded to give this information to the students attending such meetings.

The weekly teaching sessions provided an opportunity for the students to present case histories of patients they had seen and to discuss their management. Clinicians could also give a formal didactic teaching on a specific topic, for example, mental state assessment or risk assessment in psychiatry.

The letters of thanks to the participating clinicians served as an added benefit (in addition to the satisfaction of teaching others) to sustain their motivation and reward them for their contribution to the teaching of medical students. The psychiatric trainees used the letter to demonstrate their skills in teaching in their portfolio.

Benefits of the new placement structure

Helping students to feel supported before, during and even after their placements was a high priority in this project. Research has shown that learners rank the need for support and guidance in workplace environments as high and it is an essential requirement for a successful learning experience ⁽⁵⁾. This extra support is particularly crucial in psychiatry which is perceived by many students to be difficult and challenging ⁽⁶⁾.

The support provided to the students in the improved structure was in the form of having the contact details of the rota coordinators, their supervising clinicians, the administration team (medical secretaries, site manager for parking permits) and some other useful numbers for various locations and clinics.

While improving the organisation of the placement, changes were aimed to reduce the commitment of teaching and supervision for clinicians and spread it more equally among the members of the team.

Students reported that they found home visits during the placement the most useful part of their placement and the most interesting. This is an invaluable experience with the student having a significant amount of time in a one to one interaction with a clinician (including during the travel from one location to another) and then observing the clinician ‘in action’ with patients at home. This experience highlights the role of ‘professional socialisation’ ⁽⁷⁾ that is considered by educators as a significant process in the development of a sense of a shared professional identity and responsibility in both the clinician and the learner.

Furthermore, using non-medical professionals to supervise and teach students has been valued by students ⁽⁸⁾. It enriched the clinical placement with the concept of (Inter-Professional Learning) which is an active learning from and with professionals from other disciplines allied to medicine. This style of education has been shown to improve students’ communication with professionals from different disciplines and to have a better understanding of the nature of the multidisciplinary teamwork and the roles of each member of the team ⁽⁹⁾.

While improving the organisation of the placement, changes were aimed to reduce the commitment of teaching and supervision for clinicians and spread it more equally among the members of the team.

Balint groups and improving student placements in psychiatry

Balint groups were pioneered by the Hungarian psychoanalyst Michael Balint who introduced this model in the late 1950s after running seminars for general practitioners in the UK with his wife, Enid. ⁽¹⁰⁾

Balint recognised the intense emotions that affect the doctor and the patient and encouraged clinicians to talk about these feelings in groups, which later came to be known as Balint groups.

Research has shown that Balint groups for medical students can increase the students’ empathy towards patients with chronic mental illness and improve their ability to cope with complex clinical situations ⁽¹¹⁾. It also helps them to engage in reflection about their professional growth and to develop their identity as future doctors ⁽¹²⁾. Most importantly, this psychotherapeutic approach it allows them to reach a deeper understanding of the emotional impact of their patients ⁽¹³⁾. It was felt that the students would benefit from this model to help with the various emotions evoked by dealing with patients they would encounter in Old Age Psychiatry, in particular, dementia.

The student feedback was very positive for the Balint group. One student commented. It is inevitable to have experiences with patients that leave you with a feeling, whether that be positive or negative. To be able to look back at those times, talk them through, be listened to and have others reflect things back that you may not necessarily have realised yourself, is invaluable’.

Patient and carer involvement in clinical education

Clinical education in the workplace has always depended on patients and carers in its design and delivery. Students value seeing patients and learning from their experiences. However, the evidence suggests that patients are not routinely involved in the design of the curriculum or clinical placements despite calls to actively engage them in teaching and training ⁽¹⁴⁾.

Students were given the opportunity to learn from patients and carers through regular and supervised contact with them. They also attended a workshop on dementia and viewed a DVD showing the experiences of a woman with dementia and depicting how the world might be seen from her perspective. Feedback from students was very positive for these opportunities.

Medical students placement and Electro-Convulsive Therapy (ECT)

Students are allocated to spend one day in ECT clinic during their two-week placement in Old Age Psychiatry. Research has shown that many medical students have negative attitudes and unjustified reservations about ECT and its therapeutic applications ⁽¹⁵⁾. However, these views can change with education about this therapy during clinical placements and encouragement of the students to talk to patients and read about its indications and effectiveness in people with severe mental illness ⁽¹⁶⁾. Seeing the procedure first hand would, therefore, help the student gain confidence to challenge the stigma attached to ECT and to explain this treatment to their future patients.

Feedback from the students following the implementation of the placement improvement project

The feedback from medical students and clinicians was very positive. The students enjoyed their placements and felt that they gained useful knowledge and skills. Above all, they felt welcomed in the clinical settings and settled very nicely into the teams.

Figures 1 and two summarise some comments from the medical students following the placement. This feedback was collected by Norwich Medical School as part of the regular monitoring of clinical placement for medical students.

Figures 1 and 2: Feedback from students after the implementation of the changes to the clinical placement:

Limitations

There were some challenges in the implementation of this improved model. First is not always easy to recruit non-medical members of the clinical teams to take students. There are some reasons for this including lack of confidence or experience in teaching, a belief that it is “not their role”, or concern about the increasing demands on their time. Others already had students in their discipline. This was addressed by briefing the professionals about what the students need to achieve at the end of the placement and encouraging them to be involved in the supervision. The introduction of nursing revalidation in April 2016 may help more nurses to get involved. ⁽¹⁷⁾

Conclusions and recommendations

This paper describes a clinical placement improvement project for medical students in Old Age Psychiatry. The changes focused on the enhancement of organisation, supervision and teaching.

Our improvement project is ongoing, and there are areas needing further improvement, for example, more active involvement of patients and carers in the teaching and learning of medical students is necessary. It is planned to achieve this by inviting patients and carers to tell their personal stories to the students in a small group.

Organisers of students’ placement in secondary or primary care need a systematic approach to filling allocation slots to ensure that all students receive a similar and broad exposure to their speciality. It can be dispiriting and stressful to ask for volunteers constantly. They need to have good relationships with their clinical colleagues of all disciplines, and to be willing and assertive enough to go around and ask colleagues in person rather than sending email requests.

Psychiatric educators have a significant role to play in the improvement of clinical placements for students as this will hopefully contribute to improving recruitment to this medical speciality that is undergoing a recruitment crisis. Research has shown that there is a positive correlation between the length and quality of clinical placement and the likelihood of choosing psychiatry as a future career. ⁽¹⁸⁾

Teaching Points

• Despite advances in the management of ectopic pregnancies an emphasis must be given on improving the understanding of the women and the healthcare professionals of the pathophysiology of haemorrhagic shock.
• Educating the public and all health care professionals about the phrase “Think Ectopic” as a main differential in any women of childbearing age with atypical signs and symptoms of general ill health is paramount.

Précis

The significance of effective communication within multidisciplinary teams especially in emergency situations towards optimising patient care and saving lives cannot be understated.

Case Report

A 27-year-old woman who claimed to be unaware of her current pregnancy collapsed at her home. She was not known to have any co-morbidities. Paramedics were called and found her to be in cardiac arrest with pulseless electrical activity. Cardiopulmonary resuscitation (CPR) was immediately commenced. Spontaneous circulation returned after 13 minutes of CPR at home.

She was then transferred to the emergency department. On arrival to the emergency department her Glasgow Coma Scale (GCS) was 3. She had a pulse rate of 130 beats per minute; unrecordable blood pressure; haemoglobin of 55g/l; metabolic acidosis with a pH of 6.8; lactate > 15; and a potassium of 6.6 mmol/l. She was resuscitated and gradually regained consciousness with a GCS of 15.

In the midst of stabilising her condition and unaware of her pregnancy, a urine pregnancy test was obtained following siting of a urinary catheter. A positive pregnancy test prompted notification to the gynaecology team who performed ultrasonography imaging which revealed significant haemoperitoneum. An immediate decision was made to perform laparotomy in view of the most likely diagnosis of a ruptured ectopic pregnancy.

Laparotomy revealed a 3.5 litre of haemoperitoneum secondary to a ruptured right sided tubal ectopic pregnancy. A right salpingectomy was performed. The patient was subsequently transferred to the intensive care unit as her serology results were consistent with multi organ failure with a platelet count of 46 (10⁹/L); creatinine of 194 mmol/L; estimated glomerular filtration rate (egfr) of 27 mls/min/ 1.73 m²; alanine transaminase (ALT) of 441 IU/L; and alkaline phosphatase (ALP) of 49 IU/L.

She made an uneventful recovery as demonstrated in figure 1 by the improving serological parameters and was discharged home after 6 days.

Figure 1: The cumulative serology- full blood count, liver function tests, urea and electrolytes, clotting profile.ankle

Discussion

The confidential enquiry report into maternal deaths – UK has shown a decreasing trend in the case fatality rate in women with ectopic pregnancies. This has been suggested to reflect earlier detection and immediate treatment of ectopic pregnancies. However unforeseen tubal rupture with major haemorrhage continues to be a source of major morbidity and mortality. Ectopic pregnancies account for 3-4% of pregnancy related deaths. ⁴

The classical triad of symptoms encountered in ectopic pregnancy includes pain, vaginal bleeding and amenorrhoea. ¹ Worryingly, as illustrated by our case, rarely these women may present in a state of collapse even before the diagnosis of pregnancy is made. ⁴

Pathophysiology of multi-organ failure following haemorrhagic shock

Our case clearly demonstrates the detrimental multi-systemic effects and subsequent threat to life created by haemorrhage from a ruptured ectopic pregnancy. Acute haemorrhage results in decreased cardiac output and pulse pressure that is detected by baroreceptors in the aortic arch and atrium. Neural reflexes subsequently cause an increased sympathetic outflow to the heart and other vital organs resulting in vasoconstriction, and redistribution of blood flow away from non-vital organs. Neuroendocrine responses activated by neural reflexes play a major role in homeostasis during haemorrhage. Elevated aldosterone and cortisol secondary to raised adrenocorticotrophic hormone secreted by the pituitary gland leads to increased water absorption in the kidneys. The reduced tissue perfusion to non-vital organs results in insufficient delivery of oxygen and nutrients required for cellular function. ²

The resultant hypoxia leads to anaerobic metabolism and hence lactate production and metabolic acidosis. Hyperlactaemia is defined when serum lactate is greater than 4 mmol/l. ³ A level of 15mmol/l as demonstrated by our case highlights the extent of shock the patient was in.

Endogenous heat production is restricted by anaerobic metabolism, which in turns exacerbates hypothermia that is likely to be predisposed by the administration of intravenous fluids and blood products. Hypothermia is one of the reversible causes of pulseless electrical activity and a core temperature of less than 35°C is itself an independent predictor of mortality after major haemorrhage.

Furthermore, our case revealed a severe acidosis with a pH of 6.8, which is reflective of widespread cellular anaerobic respiration secondary to hypoxia as a result of inadequate perfusion. Widespread literature has shown that a pH of less than 7.2 is associated with decreased contractility, low cardiac output, bradycardia, arrhythmias and decreased blood flow to the liver and kidneys. This can lead to multi-organ failure.⁶

Many patients with severe haemorrhage can establish coagulopathy very quickly as our case has demonstrated. At present there is nouniversally established definition of coagulopathy though many experts use prolonged prothrombin time as an indicator of coagulopathy. Our case presented with a prolonged prothrombin time of 14.8 seconds. The pathophysiology is complex and stems from immediate activation of multiple haemostatic pathways including fibrinolysis, platelet and endothelium dysfunction. Furthermore, acute phase response after resuscitation measures can create a prothrombotic state. Sometimes, disseminated intravascular coagulation can occur in those who are insufficiently resuscitated or not resuscitated in a timely manner. ⁷

Effective multi-disciplinary input

This case clearly highlights that the responsibility does not solely rely on the surgeon who is required to cease the bleeding but also on the multi-disciplinary specialists including paramedics, emergency clinicians, nursing staff, anaesthetists and haematologists. This is a vital component of resuscitation management during emergency situations.

Appropriate initial fluid management

The management with intravenous fluid resuscitation remains challenging as some evidence suggests that aggressive fluid resuscitation can be detrimental because it can lead to dislodging of clots and dilutional coagulopathy leading to increased risk of haemorrhage. ⁵

Clinicians supporting this hypothesis suggest to cautiously administer fluid resuscitation with the aim of maintaining a subnormal blood pressure (systolic of 70-90 mmHg), whilst allowing sufficient oxygen delivery. The very early use of crystalloids and blood products is paramount to help treat acute coagulopathy. ⁷

Immediate surgical treatment

Recourse to immediate surgical cessation of bleeding is a vital part of the resuscitation process, and must not be delayed. ⁷ The presence of free fluid in the abdomen and a positive pregnancy test immediately alerted an ectopic pregnancy as the most likely diagnosis. The majority of women of reproductive age are free of comorbidities with a greater ability to adapt to resuscitative measures and hence showing quicker recovery.

Conclusion

Despite advances in the management of ectopic pregnancies an emphasis must be given on improving the understanding of the women and the healthcare professionals of the pathophysiology of haemorrhagic shock. Educating the public and all health care professionals about the phrase “Think Ectopic” as a main differential in any women of childbearing age with atypical signs and symptoms of general ill health is paramount.

Furthermore, the significance of effective communication within multidisciplinary teams towards optimising patient care and saving lives cannot be understated.

A 70-year-old man presented in the winter with a four-week history of redness of the left anterolateral leg. He first noticed a slight “tenderness” in the area when showering; the discomfort lasted only a few days. Over the next week, he noticed redness developing. It is now painless and not pruritic, warm, or peeling. He has not applied any topical lotions or creams. He has not had an exposure to new soaps or detergents. He feels well, without fever or weight loss. He has a diagnosis of hypertension and lumbar radiculopathy with an L5 discectomy and resultant leg numbness. He is retired and does not smoke or drink alcohol; his hobby is woodworking in his garage.

Physical examination reveals normal vital signs. On his left anterolateral leg, he has an 8 cm, irregular patch of reticulated erythema with both hyperpigmentation and scaling. The lesion is non-palpable. He has decreased sensation in an L5 distribution on that leg, which was unchanged from prior examinations. These skin findings are shown in Figure 1.

Figure 1

Question: Based on history and physical examination, which of the following is the most likely diagnosis?

• Livedo reticularis
• Erythema ab igne
• Livedo racemosa
• First-degree burn

Discussion

The answer is erythema ab igne (EAI; literally “redness from fire,”) which results from chronic exposure to moderate-intensity heat. EAI presents as a reticulated erythematous patch over the area of exposed skin. Possible secondary changes include epidermal atrophy and scaling.^1,2 With repeated exposure, brown hyperpigmentation may develop.¹ Most patients are asymptomatic, although some note a mild burning sensation. A history of repeated exposure to heat is key to the diagnosis. While cases were historically noted on skin exposed to fire, such as the arms of bakers and coal shovellers, EAI can result from our many, modern heat-sources, such as laptop computers, car seat heaters, heating pads, and, in this case, the portable space heater under the patient’s woodworking bench.^2-4 With removal of the heat source, hyperpigmentation typically regresses but may take years.^1,3 The diagnosis is clinical. A biopsy is not required to make the diagnosis, but is indicated if malignant transformation is suspected. EAI can increase risk of squamous cell carcinoma, Merkel cell carcinoma, and cutaneous marginal zone lymphoma.^1,5 Treatment is typically not necessary; topical steroids or retinoids and laser have had variable success.^1,3,4 If pre-malignant changes are detected, topical 5-flourouracil is recommended.^1,4

See Table 1 for a summary of the key characteristics and distinguishing features of each diagnosis in this selected differential.

Table 1. Selected Differential Diagnosis of Reticulated Skin Lesions in Adults

Livedo reticularis is typically more violaceous in appearance, with net-like, mottled discolouration of the skin due to deoxygenation and dilation of the venous plexus. Primary, physiologic livedo reticularis is often brought on by cold and alleviated by warming. It usually involves a larger area, such as the bilateral thighs, rather than a confined area of skin.^1,2

Livedo racemosa is a persistent variant of livedo reticularis with a characteristic, large, broken, branching pattern, often on the trunk and proximal limbs. It is generally secondary to a systemic disease, such as antiphospholipid antibody syndrome or Sneddon syndrome.⁶

First-degree burns are erythematous, dry, and painful. Instead of a reticulated pattern, as shown here, the erythema of first degree burns covers the entire area of skin that contacted the high-intensity heat source.

Introduction

Spasticity was first described by Lance¹ in 1980, and according to him it was described as; a motor disorder characterised by a velocity-dependent increase in tonic stretch reflexes (muscle tone) with exaggerated tendon jerks, resulting from hyperexcitability of the stretch reflex, as one component of the upper motor neuron syndrome. Spasticity can be a consequence of many neurological conditions including traumatic brain injury, spinal cord injury, stroke and multiple sclerosis. The annual incidence of spasticity in the lower limb following a stroke, traumatic brain injury and spinal cord injury is estimated to be 30 to 485 per 100,000, 100-235 per 100,000 and 0.2 to 8 per 100,000 respectively². Spasticity is characterised by muscle overactivity and can lead to permanent changes in the muscle fibres leading to muscle contractures. Contractures can be very painful and may interfere with seating, posture, mobility and activities of daily living, thus increasing the care cost significantly.

Phenol has been used peripherally and intrathecally for the treatment of spasticity for many years. The botulinum toxin became available in the last decade for treatment of spasticity. Its use has increased since then, and this has led to a decline in the use of phenol. It is still being used in patients who are sensitive to botulinum toxins or have developed antibodies to them. Phenol is both neurolytic and anaesthetic in nature³. The anaesthetic effect of phenol can be seen immediately after the injection where the patient reports an immediate effect. The neurolytic effect takes at least two weeks, and therefore patients should be educated not to expect any significant change in the spasticity before two to four weeks. Phenol can also be used in combination with botulinum toxin to treat multifocal spasticity where the maximum dose of botulinum exceeds the recommended safe dose. This allows several groups of muscles to be treated in a single setting³.

The lethal dosage of phenol has been reported to be greater than eight grams⁴. Phenol in aqueous solution is preferred for peripheral nerve and motor point blocks and is available in 5, 6 and 7% concentration. Injecting botulinum toxins is quite different from performing nerve and motor point blocks. Phenol nerve and motor point blocks take a longer duration of time to perform as compared to botulinum toxins. For motor point blocks, a nerve stimulator with a surface electrode is needed to localise the motor points on the muscles. In the present study, we highlight the importance of management of spasticity in adults with a combination of botulinum toxin and phenol nerve /motor point blocks. A case series of patients who underwent combined phenol and botulinum toxin is presented, describing the diagnosis, number and location of muscles injected, types of phenol nerve and motor point blocks and any complications encountered.

Methods

This is a retrospective study conducted at the Rehabilitation Medicine Department of the University Hospital in Cambridge UK. The study period included from December 2014 to January 2017. The patients were identified from the spasticity clinic database. All patients were assessed in the spasticity clinic, and a plan to inject the botulinum toxin along with phenol nerve block/motor point block agreed with the patient. The patients who decided to have the procedure were appointed to a clinic to perform the agreed injections and blocks. If the patients were on anticoagulants (warfarin, dalteparin or clopidogrel), they were advised to stop the anticoagulation 3 days before the procedure. International Normalised Ratio (INR) was checked before the procedure. The usual dose of anticoagulation was started after the procedure.

Patients were consented and placed on a plinth. Botulinum toxin type A only was used in our study. It was diluted with normal saline, and the muscles were injected either using the surface anatomy or electrical stimulation. Each muscle was either injected at one to two sites, depending on the size of the individual muscle.

Phenol nerve blocks and motor point blocks were performed according to the techniques described by Roy³ and Gaid⁵. Aqueous phenol 5% (phenol in water) was used for all the procedures. The nerves were identified using a nerve stimulator with a surface electrode using 2mA current (Figure 1). The skin was infiltrated with 1% lignocaine, and the nerve was approached with a stimulator needle. The nerve was then ablated with 5% phenol under stimulation guidance. The dose of the phenol was titrated while the nerve was being stimulated. The motor points were located similarly with the help of a surface electrode and marked before ablation with 1 to 2 ml of 5% phenol. The amount of botulinum toxin and phenol was recorded. All patients were reviewed in 6 weeks’ time for any complications.

Figure 1: Nerve Stimulator with surface electrode

Results

Between December 2014 to January 2017, we treated 29 patients with spasticity caused by different neurological conditions with a combination of aqueous phenol and botulinum toxin injections. There were 15 males and 14 females with an age range of 18 to 80 years and a mean age of 49.3 years. The most common diagnosis was multiple sclerosis followed by stroke (Figure 2). A total of 40 phenol nerve or motor point blocks were performed in 29 patients. Nineteen patients (65.5%) received phenol blocks once, 9 (31%) twice and only 1 patient (3.4%) had the phenol block done three times. Where the phenol blocks were repeated, the mean duration between the phenol injection was 14.1 months (range 6-23 months). The procedure was bilateral in 16 (55.2%) and unilateral in 13 (44.8%). The local anaesthetic (trial block) was performed in 6 (20.6%) patients who were ambulatory before the phenol block.

Figure 2: Frequency of Diagnosis

Obturator nerve block was the most common phenol procedure performed (44.8%), followed by posterior tibial nerve block (37.9%). Two (6.9%) patients had both obturator nerve blocks and posterior tibial nerve blocks, whereas 1(3.4%) patient had hamstring motor point blocks, 1(3.4%) patient had gastrocnemius motor point blocks. One patient (3.4%) had bilateral obturator nerve blocks, posterior tibial nerve blocks and rectus femoris motor point blocks (Figure 3).

Figure 3: Frequency of Phenol Nerve/Motor Point Blocks

Botulinum toxin was also injected into various muscles in all 29 patients. The botulinum was repeated every 4 to 6 months in the same muscles. Botulinum toxins were injected bilaterally in 12 (41.4%) and unilaterally in 17 (63.6%) patients. The most common muscles injected with botulinum toxin were hamstrings (44.8%) followed by finger flexors (13.8%). The frequency of botulinum toxins injections is shown in Figure 4.

Figure 4: Muscles Injected with Botulinum Toxins

The most common combination in our series was obturator nerve block and hamstring botulinum toxin injections (34.4%). The combination of posterior tibial nerve block with hamstring botulinum toxins was used in 3 (10.3%), and 2 (6.8%) patients received posterior tibial nerve block with finger flexor botulinum toxin injections. The combination of phenol and botulinum toxin injection is shown in Table 1. There were no complications noted following both phenol as well as botulinum toxin injections.

Table 1: Combination of Phenol and Botulinum Toxins used

Discussion

Perineural injection of aqueous phenol (3 to 7%) can reduce spasticity by blocking the nerve signals to the group of muscles supplied by the nerve. Phenol produces an initial local anaesthetic effect which is followed by neurolysis caused by protein coagulation and inflammation⁶. The neurolysis leaves the nerve with about 25% less function than before but does not disadvantage people with little or no residual function, as a mild progressive denervation can be beneficial in reducing spasticity⁶. Khalili et al⁷first described the technique of phenol nerve blocks and also suggested that the re-growth of most axons is seen with preservation of gamma motor neurons. This means that phenol reduces spasticity without reducing the strength of the muscle significantly.

The use of combining phenol with botulinum toxins injections has been documented in children with cerebral palsy and central nervous degenerative diseases⁸. To date, there are no studies in the literature showing the use of combined phenol and botulinum toxins in the treatment of spasticity in adults. The combination of phenol with botulinum toxin helps to treat multifocal spasticity allowing more spastic areas to be treated. The most frequent pattern used in Gooch et al⁸ study was obturator nerve block and gastrocnemius botulinum toxin injections. In our study, the most common combination was obturator nerve block and hamstring botulinum toxin injections. The possible explanation for this variance is that the majority of our study population suffered from multiple sclerosis and hamstring with hip adductor spasticity is a very common pattern.

The mechanism of action of phenol is different from botulinum toxins. However, the reduction in spasticity with phenol and botulinum toxins is comparable. Manca et al⁹ compared botulinum toxins and phenol nerve blocks to reduce ankle clonus in spastic paresis and concluded that both patient groups showed significant clonus reduction over time with the phenol group effect greater than the botulinum toxins group. They also suggested that the two drugs have a different mechanism of action with phenol reducing the excitability of the alpha motor neuron. A randomised double-blind trial by Kirazli et¹⁰ al compared the effects of botulinum toxins Type A and phenol on post-stroke ankle plantar flexor and invertor spasticity. There was a significant change in Ashworth scores at week 2 and 4 in the group who received botulinum toxins but there was no significant difference between the two groups at week 8 and 12¹⁰. Similarly, the decrease in clonus duration (detected by electromyography) was significant in both groups. However, the group that received botulinum toxins showed significant change at week 2 and 4 compared to phenol group. The reason for this may be the delayed onset of action of phenol as compared to botulinum toxins. Burkel et al⁶ studied the effects of phenol into the peripheral nerves of rats and showed that Wallerian degeneration of the nerves occurs before healing by fibrosis that starts after about 4-6 months following phenol injections. Their study also concluded that following phenol the nerves are left with 25% less function than before and this does not disadvantage the people with little or no residual function⁶.

There is always a risk of deteriorating the mobility or function due to weakness caused by the phenol nerve block. It is our usual practice to perform a local anaesthetic block (trial block) before injecting the phenol in all ambulatory patients or patients who are using spasticity functionally to their advantage. In our series, 20.6% of patients underwent local anaesthetic block before proceeding to the phenol block. There were no adverse effects noted following the local anaesthetic block, and all six patients chose to have the phenol blocks. A recent study by McCrea et al¹¹ looked at the effects of phenol on position and velocity components of spasticity in addition to strength in post-stroke elbow flexor spasticity. The study concluded that phenol paradoxically improved muscle strength in addition to reducing hypertonia¹¹.

In our series, we used phenol mainly for lower limb muscles and botulinum toxins for both the lower and upper limb muscles. For smaller muscles of the upper limb, it is difficult, but not impossible, to find the motor points. The technique for upper limb phenol blocks has been well described in literature³. However, when combining the botulinum toxins with phenol, we find it useful to prefer the phenol block for the lower limb muscles. Gooch et al⁸ also injected larger proximal muscles with phenol, and smaller distal and deeper muscles with botulinum toxins. In our series, the maximum dose of botulinum toxins used was 1000 units of Dysport and the maximum dose of phenol used was 20 ml of 5% aqueous phenol.

Conclusion

The combination of botulinum toxin with phenol injections is effective in treating multi-focal spasticity in clinical settings. The advantage of using phenol in combination with botulinum toxins is cost-reduction and the flexibility of managing various muscle groups at the same time. Further studies are needed to evaluate the long-term cost-effectiveness and complications of combining phenol and botulinum toxins, especially after repeated injections.

Background

Sjögren’s syndrome (SS) is an autoimmune exocrinopathy characterised by a lymphoplasmacytic infiltration of the exocrine glands. Both xerophthalmia and xerostomia are the most common manifestations of the disease. However serious organ damage such as, pulmonary and neurological involvement, can occur. The prevalence of neurological manifestations of SS varies between 0% and 70% (average 20%), which is largely dominated by peripheral neuropathies¹. The cranial nerve involvement, especially when it is isolated, represents a rare facet of the peripheral neuropathy.

Observation

We report the case of a 62-year-old patient with no medical history, referred to the internal medicine department with a 6 years history of dry mouth and xerophthalmia. No other complaints were reported.

The mouth examination showed a fissured, smooth and left deviated tongue without evidence of atrophy or fasciculation (figure 1). The rest of the oropharyngeal examination was unremarkable with no angina or cervical lymphadenopathy. Neurological examination confirmed a deficit of the right XII cranial nerve and excluded other cranial nerve involvements, sensibility or motility deficit. A specialised ophthalmologic examination was performed and showed a bilateral superficial punctuate keratitis.

The search for antinuclear antibodies by indirect immunofluorescence was positive at the titre of 1/1280 (speckled) corresponding to Anti-SSA and Anti-SSB antibodies. Cryoglobulinemia search was negative.

The rest of the laboratory investigations (blood cell count, liver and renal function tests, thyroid balance and inflammation markers) were normal.

A labial salivary gland biopsy was performed and its histological examination showed a lymphoid cell cluster of more than 50 cells/ 4 mm² corresponding to a focus score 1.

Brain MRI was normal - no damage in the brain stem was seen. Electromyography was normal.

The diagnosis of SS was made according to the presence of five out of six criteria according to the European American study group. The diagnosis of primary SS was retained due to the lack of clinical or biological argues for an associated autoimmune disease. A symptomatic treatment of Sicca syndrome was prescribed but no specific therapy has been initiated for the hypoglossal nerve attempt because of its asymptomatic nature.

Discussion

In the case of our patient, the tongue deviation was discovered at physical examination and was totally asymptomatic. In other cases, the twelfth nerve palsy could be responsible for swallowing difficulties, and in advanced stages for a lingual or hemi lingual amyotrophy. The spectrum of its aetiologies is numerous. In a large case series of 100 patients, malignant tumors (about half of cases), neurological causes (16 %) and post-traumatic palsy (12% of cases) were the three most popular aetiologies². Other conditions could be associated with twelfth nerve palsy, such as, infections², vascular injury³ and non-invasive oxygen therapy⁴. Paroxysmal idiopathic hypoglossal nerve palsy has also been described⁵. Our patient had Sicca syndrome which was related to SS according to 5 criteria of the European American study group: it was a subjective sensation of dry mouth and dry eyes associated to a bilateral punctuated keratitis, a focus score > 1 at the histological examination of the salivary gland biopsy and positive anti-SSA and anti-SSB⁶.

SS is an autoimmune disease that often presents as dry eyes and dry mouth due to lacrimal and salivary gland involvement. It can be primitive or associated to other autoimmune diseases such as, Hashimoto’s Thyroiditis, Rheumatoid Arthritis or Systemic Lupus Erythematosus. Wide varieties of neurological complications are characteristic features of SS which occurs most frequently in the primary form. Peripheral neuropathy is the most frequent neurological manifestation. Its most common presentation is a symmetrical sensorimotor or pure sensory neuropathy of hands and feet. Sensitive neuropathy, small fiber neuropathy, multiple mononeuropathy and polyradiculoneuropathy have also been described¹. Cranial nerve involvement is rare. In a review of the literature, Colaci M found 267 patients suffering from SS with different types of cranial neuritis during their clinical history. The discovery of cranial neuritis was contemporary to SS diagnosis in 40% of the patients, as in the case of our patient.

Optic neuritis and trigeminal nerve injury were the most frequent attempts and represent respectively 46.4% and 38% of all cranial nerve palsies. All cranial nerves palsies have been described except the eleventh⁷. Involvement of the twelfth cranial nerve is very rare and only two cases have been described⁸′ ⁹. In these two cases, it was associated with an involvement of other cranial nerves (table 1). To the best of our knowledge, this is the first report of an isolated and permanent involvement of the twelfth cranial nerve in a patient with primary SS. Many mechanisms were proposed to explain the cranial nerve involvement in SS. Clinicopathological observations of Mori K⁸ suggest that an isolated trigeminal nerve attempt could be explained by an immune-mediated neuron death in the sensory gasser ganglion. Whereas, other cranial nerve involvements which are frequently associated together could be explained by a multiple mononeuropathy resulting from a vasculitis⁸.

Further clinical observations will be necessary to determine the exact mechanisms of such neurological involvement.

Table 1: Review of the literature regarding SS patients with hypoglossal nerve injury

Figure 1: Smooth and left deviated tongue

Conclusion

In front of cranial nerves neuritis, we should actively search for sicca syndrome, sometimes not spontaneously reported by patients. Examination of the mouth can be instructive and should not be omitted in the diagnosis and monitoring of Sjögren’s syndrome.